Publications by authors named "Heidi M Connolly"

253 Publications

Catheter Ablation in Patients With Neuroendocrine (Carcinoid) Tumors and Carcinoid Heart Disease: Outcomes, Peri-Procedural Complications, and Management Strategies.

JACC Clin Electrophysiol 2021 Feb 29;7(2):151-160. Epub 2020 Oct 29.

Division of Cardiovascular Disease, Department of Medicine, Mayo Clinic, Rochester, Minnesota, USA. Electronic address:

Objectives: This report describes a series of patients with neuroendocrine tumors with or without carcinoid heart disease undergoing catheter ablation at the authors' institution.

Background: Neuroendocrine (carcinoid) tumors are a rare form of neoplasm with the potential for systemic vasoactive effects and cardiac valvular involvement. These tumors can create peri-operative management challenges for the electrophysiologist. However, there are few data regarding ablation outcomes, periprocedural complications, and management of these patients.

Methods: All patients with neuroendocrine tumors undergoing catheter ablation at the Mayo Clinic, Rochester, Minnesota over a 25-year period were retrospectively reviewed. From this cohort, the type of arrhythmias ablated, the recurrence of arrhythmia, perioperative complications, and mortality were reviewed and analyzed.

Results: A total of 17 patients (52.9% male; mean age 62.4 ± 9.3 years) with neuroendocrine tumors underwent catheter ablation during the study period. Primary tumor sites included the gastrointestinal tract (n = 11), lung (n = 4), ovary (n = 1), and lymph node (n = 1). Nine patients had metastatic disease, 5 of whom were on somatostatin analog therapy at the time of ablation. Three patients had active symptoms of carcinoid syndrome at the time of ablation, and 2 of those patients had carcinoid heart disease. Ablations were performed mainly for atrial arrhythmias (76.5%): atrioventricular nodal re-entry tachycardia (n = 7), atrial fibrillation (n = 4), and atrial flutter (n = 2). Four patients underwent ablation of ventricular arrhythmias. During a mean follow-up of 19.2 ± 26.2 months, arrhythmia recurred in 35.3% of patients. Three patients (17.6%) had periprocedural complications: pericardial effusion (n = 1), groin site hematoma (n = 1), and carcinoid crisis (n = 1). No deaths were noted in the peri-operative period.

Conclusions: In a unique cohort of patients with neuroendocrine tumors, catheter ablation was feasible in patients with or without carcinoid syndrome. Carcinoid crisis may occur during the periprocedural period, which can be life-threatening, and a specified protocol for management is important to mitigate this risk.
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http://dx.doi.org/10.1016/j.jacep.2020.08.009DOI Listing
February 2021

Hemodynamics and Clinical Implications of Occult Left Ventricular Dysfunction in Adults Undergoing Ebstein Anomaly Repair.

Circ Cardiovasc Imaging 2021 Feb 15;14(2):e011739. Epub 2021 Feb 15.

Department of Cardiovascular Medicine, Mayo Clinic, Rochester, MN.

Background: Left ventricular global longitudinal strain (LVGLS) can detect early phases of LV systolic dysfunction, but its application has not been studied in Ebstein anomaly. We hypothesized that LVGLS can detect early phases of LV systolic dysfunction and that patients with occult LV systolic dysfunction will have worse hemodynamics, end-organ dysfunction, and suboptimal postoperative LV reverse remodeling after tricuspid valve surgery in comparison to patients with normal LV systolic function.

Methods: In this retrospective cohort study, 371 Ebstein patients that underwent tricuspid valve surgery were divided into 3 groups: normal LV systolic function (normal LVGLS and LV ejection fraction; n=244, 77%), occult LV systolic dysfunction (abnormal LVGLS with normal LV ejection fraction; n=44, 14%), and overt LV systolic dysfunction (abnormal LVGLS and LV ejection fraction; n=27, 9%).

Results: Compared with the normal LV function group, the occult group had smaller LV volume and cardiac output (2.1±0.4 versus 2.9±0.6 L/min per m, <0.001), worse end-organ dysfunction (glomerular filtration rate, 78±14 versus 91±18 mL/min per 1.73 m, =0.01), and suboptimal postoperative LV reverse remodeling. Although both the occult and overt groups had a similar degree of end-organ dysfunction (glomerular filtration rate, 78±14 versus 82±16 mL/min per 1.73 m, =0.3), the occult group was less likely to be on heart failure therapy (48% versus 96%, <0.001).

Conclusions: Abnormal LVGLS was associated with suboptimal postoperative LV reverse remodeling. These data suggest that LVGLS can potentially be used for risk stratification and provides a foundation for further studies to determine whether optimal heart failure therapy or tricuspid valve intervention can improve outcomes for LV systolic dysfunction in patients with Ebstein anomaly.
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http://dx.doi.org/10.1161/CIRCIMAGING.120.011739DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7888701PMC
February 2021

A Comparison of Hemodynamic and Clinical Outcomes After Transcatheter Versus Surgical Therapy in Adults in Coarctation of Aorta.

J Invasive Cardiol 2021 Mar 11;33(3):E191-E199. Epub 2021 Feb 11.

Mayo Clinic and Foundation, 200 First Street SW, Rochester, MN 55905 USA.

Background: Transcatheter stent therapy provides similar acute reduction in coarctation of aorta (COA) gradient and systolic blood pressure (SBP) as compared with surgery. However, there are limited data comparing mid-term outcomes after transcatheter vs surgical therapy for COA. The purpose of this study was to compare temporal changes in Doppler COA gradient and SBP after transcatheter stent therapy versus surgical therapy for COA.

Methods: A retrospective study of COA patients (≥18 years old) undergoing transcatheter stent therapy or surgical therapy at Mayo Clinic in Rochester, Minnesota from 2000-2018 was performed. Linear regression analyses were used to compare temporal changes in Doppler gradient and SBP between the 2 groups. Propensity matching was used to adjust for between-group differences in clinical and anatomic characteristics.

Results: A total of 44 and 128 patients underwent transcatheter and surgical therapy, respectively; there were no significant between-group differences in the anatomy of the thoracic aorta. Both groups had similar acute reduction in Doppler peak gradient (P=.66), mean gradient (P=.41), SBP (P=.22), and upper-to-lower extremity SBP (ULE-SBP) gradient (P=.69). The median follow-up was 46 months (interquartile range, 27-81 months) and 63 months (interquartile range, 41-94 months) in the transcatheter and surgical groups, respectively. There were no significant between-group differences in the temporal change in Doppler peak gradient (P-interaction=.06), mean gradient (P-interaction=.15), SBP (P-interaction=.20), and ULE-SBP gradient (P-interaction=.51).

Conclusions: These favorable short- and mid-term outcome data support the use of transcatheter therapy as an alternative to surgery in adults with COA. Further studies are required to determine if these favorable outcomes are maintained on long-term follow-up.
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March 2021

Long-Term Survival of Patients With Left Ventricular Noncompaction.

J Am Heart Assoc 2021 Jan 14;10(2):e015563. Epub 2021 Jan 14.

Department of Cardiovascular Diseases Mayo Clinic Rochester MN.

Background The prognosis of left ventricular noncompaction (LVNC) remains elusive despite its recognition as a clinical entity for >30 years. We sought to identify clinical and imaging characteristics and risk factors for mortality in patients with LVNC. Methods and Results 339 adults with LVNC seen between 2000 and 2016 were identified. LVNC was defined as end-systolic noncompacted to compacted myocardial ratio >2 (Jenni criteria) and end-diastolic trough of trabeculation-to-epicardium (X):peak of trabeculation-to-epicardium (Y) ratio <0.5 (Chin criteria) by echocardiography; and end-diastolic noncompacted:compacted ratio >2.3 (Petersen criteria) by magnetic resonance imaging. Median age was 47.4 years, and 46% of patients were female. Left ventricular ejection fraction <50% was present in 57% of patients and isolated apical noncompaction in 48%. During a median follow-up of 6.3 years, 59 patients died. On multivariable Cox regression analysis, age (hazard ratio [HR] 1.04; 95% CI, 1.02-1.06), left ventricular ejection fraction <50% (HR, 2.37; 95% CI, 1.17-4.80), and noncompaction extending from the apex to the mid or basal segments (HR, 2.11; 95% CI, 1.21-3.68) were associated with all-cause mortality. Compared with the expected survival for age- and sex-matched US population, patients with LVNC had reduced overall survival (<0.001). However, patients with LVNC with preserved left ventricular ejection fraction and patients with isolated apical noncompaction had similar survival to the general population. Conclusions Overall survival is reduced in patients with LVNC compared with the expected survival of age- and sex-matched US population. However, survival rate in those with preserved left ventricular ejection fraction and isolated apical noncompaction was comparable with that of the general population.
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http://dx.doi.org/10.1161/JAHA.119.015563DOI Listing
January 2021

Prognostic Role of Hepatorenal Function Indexes in Patients With Ebstein Anomaly.

J Am Coll Cardiol 2020 12;76(25):2968-2976

Department of Cardiovascular Medicine, Mayo Clinic, Rochester, Minnesota, USA.

Background: Hepatorenal dysfunction is a risk factor for mortality in patients with chronic tricuspid regurgitation due to acquired heart disease. Ebstein anomaly is the most common cause of primary tricuspid regurgitation in adults with congenital heart disease, but the prevalence and prognostic implications of hepatorenal dysfunction are unknown in this population.

Objectives: The purpose of this study was to determine the risk factors and prognostic implications of hepatorenal dysfunction, as measured primarily by the use of model for end-stage liver disease excluding international normalized ratio (MELD-XI score), as well as looking at other associated factors.

Methods: This was a retrospective study of adults with Ebstein anomaly who received care at Mayo Clinic from 2003 to 2018.

Results: Of 692 patients, the median MELD-XI score was 10.2 (interquartile range: 9.4 to 13.3); 53 (8%) died and 3 (0.4%) underwent heart transplant. MELD-XI was an independent predictor of death/transplant (hazard ratio: 1.32; 95% confidence interval: 1.11 to 2.06; p < 0.001). In the subset of patients with serial MELD-XI scores (n = 416), temporal change in MELD-XI score (ΔMELD-XI) was also a predictor of death/transplant. In the subset of patients who underwent tricuspid valve surgery (n = 344), a post-operative improvement in MELD-XI score (ΔMELD-XI) was associated with improved long-term survival. Impaired right atrial (RA) reservoir strain and elevated estimated RA pressure were associated with worse baseline MELD-XI and ΔMELD-XI scores.

Conclusions: Hepatorenal dysfunction is a predictor of mortality in Ebstein anomaly, and RA dysfunction and hypertension are hemodynamic biomarkers that can identify patients at risk for deterioration in hepatorenal function and mortality. These data highlight the prognostic importance of noncardiac organ-system dysfunction, and provide complementary clinical risk stratification metrics for management of these patients.
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http://dx.doi.org/10.1016/j.jacc.2020.10.035DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7755072PMC
December 2020

Prevalence of pulmonary hypertension in adults after atrial switch and role of ventricular filling pressures.

Heart 2020 Oct 7. Epub 2020 Oct 7.

Department of Cardiovascular Diseases, Mayo Clinic, Rochester, Minnesota, USA.

Objective: To assess the prevalence of elevated systemic right ventricular (sRV) end-diastolic pressure and pulmonary arterial hypertension in adults with transposition of the great arteries (TGA) who have undergone atrial switch operation.

Methods: Forty-two adults (aged ≥18 years) with complete TGA and atrial switch palliation undergoing cardiac catheterisation between 2004 and 2018 at Mayo Clinic, MN, were identified. Clinical, echocardiographic and invasive haemodynamic data were abstracted from the medical charts and procedure logs.

Results: Mean age was 37.6±7.9 years; 28 were male (67%). The Mustard operation was performed in 91% of individuals. Mean estimated sRV ejection fraction by echocardiography was 33.3%±10.9% and ≥moderate tricuspid (systemic atrioventricular valve) regurgitation was present in 15 patients (36%). Mean sRV end-diastolic pressure was 13.2±5.4 mm Hg. An sRV end-diastolic pressure >15 mm Hg was present in 35% of individuals whereas a pulmonary artery wedge pressure (PAWP) >15 mm Hg was seen in 59%. Mean pulmonary artery pressure ≥25 mm Hg was seen in 47.5% of patients with PAWP being >15 mm Hg in all but one patient.

Conclusion: In adults after atrial switch, elevated sRV end-diastolic pressure was present in only one-third of patients whereas increased PAWP was seen in almost 60%. These findings are most likely related to a combination of decreased pulmonary atrial (functional left atrium) compliance and, in a subset of patients, pulmonary venous baffle obstruction. Elevation in pulmonary pressures was highly prevalent with concomitant elevation in PAWP being present in essentially all patients.
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http://dx.doi.org/10.1136/heartjnl-2020-317111DOI Listing
October 2020

Predictors of left ventricular reverse remodelling after coarctation of aorta intervention.

Eur Heart J Cardiovasc Imaging 2020 Oct 6. Epub 2020 Oct 6.

Department of Cardiovascular Medicine, Mayo Clinic, Rochester, MN 55905, USA.

Aims: Several coarctation of aorta (COA) severity indices are used for timing of COA intervention, and to define severity of residual coarctation post-intervention. However, it is unclear how many of these COA indices are required in order to recommend intervention, and what degree of residual coarctation results in suboptimal recovery of the left ventricle (LV). Our aim was to assess the correlation between different COA indices and effects of chronic LV pressure overload (LV hypertrophy, diastolic, and systolic dysfunction), and to determine the effect of residual coarctation on LV reverse remodelling after COA intervention.

Methods And Results: COA severity indices were defined as Doppler COA gradient, systolic blood pressure (SBP, upper-to-lower-extremity SBP gradient, aortic isthmus ratio. LV remodelling indices were defined as LV mass index (LVMI), LV global longitudinal strain (LVGLS), e' and E/e'. LV reverse remodelling was defined as the difference between indices obtained pre-intervention and 5-year post-intervention (delta LVMI, e', E/e', LVGLS).Of the COA indices analysed in 546 adult COA patients, aortic isthmus ratio had the strongest correlation with LVMI (β ± standard error -28.3 ± 14.1, P < 0.001), LVGLS (1.51 ± 0.42, P = 0.005), e' (3.11 ± 1.10, P = 0.014), and E/e' (-13.4 ± 6.67, P = 0.008). Residual aortic isthmus ratio also had the strongest correlation with LV reverse remodelling, and residual aortic isthmus ratio <0.7 was predictive of suboptimal LV reverse remodelling post-intervention.

Conclusion: Considering the known prognostic implications of LV remodelling and reverse remodelling in response to pressure overload, these results support the use of aortic isthmus ratio for timing of COA intervention, and for prognostication post-intervention.
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http://dx.doi.org/10.1093/ehjci/jeaa199DOI Listing
October 2020

Repair of Partial Atrioventricular Septal Defects in Adults: A Single Center Experience.

Semin Thorac Cardiovasc Surg 2020 Aug 26. Epub 2020 Aug 26.

Department of Cardiovascular Surgery, Mayo Clinic, Rochester, Minnesota.

Limited data are available on long-term outcome after repair of partial atrioventricular septal defects (pAVSD) in adults. We sought to review our experience. Between January 1, 1957 and December 31, 2016, 179 adult patients [median age (IQR) 34 (18, 72) years] underwent primary repair of pAVSD. The most common associated defects were secundum atrial septal defect (n = 38) and ventricular septal defect (VSD) (n = 7). Left atrioventricular valve (LAVV) zone of apposition (ZOA) was complete in 47 patients and LAVV regurgitation (≥moderate) was present in 73 patients. Autologous pericardium (n = 79, 45%) and polytetrafluoroethylene felt (n = 56, 32%) were mainly used for pAVSD closure.  Repair techniques for LAVV regurgitation included: ZOA suture closure (n =  142), suture annuloplasty (n = 10) and posterior band annuloplasty (n = 9). Six had LAVV replacement. There were 61 deaths over a median follow-up of 21 years (IQR 10, 38), with only 4 early deaths. In a limited subset of patients with 80 paired measurements (n = 40), median right ventricular systolic pressure declined from 43 mm Hg (IQR 35-51) to 33 mm Hg (IQR 30-44) postoperatively (P < 0.001), and this improvement was sustained over long-term follow-up (P = 0.513). A total of 34 patients underwent a reoperation (recurrent LAVV regurgitation, n = 26; left ventricular outflow-tract obstruction, n = 7; LAVV stenosis, n = 4; patch dehiscence, n = 1) with cumulative incidence of 6% and 16% at 10 and 15 years, respectively. Repair of pAVSD in adults can be done safely with low early mortality and good long-term outcomes. Postrepair reduction of pulmonary artery pressure is significant. Despite the low re-operation rates, long-term surveillance remains essential.
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http://dx.doi.org/10.1053/j.semtcvs.2020.08.009DOI Listing
August 2020

Hepatocellular carcinoma and the Fontan circulation: Clinical presentation and outcomes.

Int J Cardiol 2021 Jan 21;322:142-148. Epub 2020 Aug 21.

Department of Pediatrics, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA; Adult Congenital Heart Disease, Heart Centre, King Faisal Specialist Hospital and Research Centre, Riyadh, Saudi Arabia. Electronic address:

Background: Fontan-associated liver disease (FALD) is universal in patients with a Fontan circulation. Hepatocellular carcinoma (HCC) is one of its severe expressions, and, though rare, frequently fatal. The purpose of this study was to describe the clinical presentation, risk factors, and outcomes of HCC in patients with a Fontan circulation.

Methods: A multicenter case series of Fontan patients with a diagnosis of HCC formed the basis of this study. The case series was extended by published cases and case reports. Clinical presentation, tumor characteristics, laboratory and hemodynamic findings as well as treatment types and outcomes, were described.

Results: Fifty-four Fontan patients (50% female) with a diagnosis of HCC were included. Mean age at HCC diagnosis was 30 ± 9.4 years and mean duration from Fontan surgery to HCC diagnosis was 21.6 ± 7.4 years. Median HCC size at the time of diagnosis was 4 cm with a range of 1 to 22 cm. The tumor was located in the right hepatic lobe in 65% of the patients. Fifty-one percent had liver cirrhosis at the time of HCC diagnosis. Fifty percent of the patients had no symptoms related to HCC and alpha-fetoprotein was normal in 26% of the cases. Twenty-six patients (48%) died during a median follow-up duration of 10.6 (range 1-50) months.

Conclusions: HCC in Fontan patients occurs at a young age with a 1-year survival rate of only 50%. Meticulous liver surveillance is crucial to detect small tumors in the early stage.
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http://dx.doi.org/10.1016/j.ijcard.2020.08.057DOI Listing
January 2021

Hemodynamics in Adults With the Shone Complex.

Am J Cardiol 2020 09 16;130:137-142. Epub 2020 Jun 16.

Department of Cardiovascular Medicine, Mayo Clinic, Minnesota. Electronic address:

Patients with Shone complex (SC) have multiple left-sided obstructive lesions and thus are at risk for left ventricular (LV) remodeling, LV diastolic dysfunction and pulmonary hypertension. Yet, to date, there has been no description of hemodynamics in adults with SC. Retrospective chart review of 25 patients with SC who underwent cardiac catheterization at Mayo Clinic, MN between 2002 and 2019 was performed. SC was defined as multiple left-sided obstructive lesions in the presence of an anatomically abnormal mitral valve. Median age was 32 years (22.5, 42) and 15 patients (60%) were female. The majority of patients (84%) had history of coarctation of the aorta, 10 (40%) had subaortic stenosis, 11 (44%) had prior aortic valve replacement, and 10 (40%) had prior mitral valve replacement. Structural disease at the time of catheterization which warranted intervention within the next year was present in 13 patients (52%). The mean LV end-diastolic pressure was 21.3 ± 9.0 mm Hg (>15 mm Hg in 71%), pulmonary artery peak systolic pressure was 55.4 ± 13.4 mm Hg, and the pulmonary artery mean pressure was 37.0 ± 9.4 mm Hg (>20 mm Hg in 96%). During a mean follow-up of 8.3 ± 4.4 years, there were 7 deaths (28%) and 3 additional patients (12%) underwent cardiac transplantation. In conclusion, adults with SC who underwent catheterization showed significant left-sided heart and pulmonary vascular remodeling. Elevated LV end-diastolic pressure and pulmonary artery pressures were highly prevalent. There were high mortality and cardiac transplant rates in our cohort.
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http://dx.doi.org/10.1016/j.amjcard.2020.06.024DOI Listing
September 2020

Ebstein Anomaly in the Adult Patient.

Cardiol Clin 2020 Aug 6;38(3):353-363. Epub 2020 Jun 6.

Department of Cardiovascular Medicine, Mayo Clinic, 200 First Street Southwest, Rochester, MN 55905, USA.

Ebstein anomaly is a congenital malformation involving primarily the tricuspid valve, with failure of delamination from the underlying myocardium and right ventricular myopathy. Echocardiography is diagnostic in most patients and demonstrates apical displacement of the septal leaflet and variable tethering of leaflet tissue to the right ventricular myocardium. Operative intervention is considered for exertional symptoms, progressive right ventricular enlargement, or right ventricular dysfunction. Tricuspid valve cone repair is the preferred surgical approach. Tricuspid valve replacement and bidirectional cavopulmonary shunt also are considered in patients with advanced disease. Pregnancy generally is well tolerated. Patients with Ebstein anomaly require lifelong follow-up.
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http://dx.doi.org/10.1016/j.ccl.2020.04.004DOI Listing
August 2020

Prognostic implications of inferior vena cava haemodynamics in ambulatory patients with tetralogy of Fallot.

ESC Heart Fail 2020 10 26;7(5):2589-2596. Epub 2020 Jun 26.

Department of Cardiovascular Medicine, Mayo Clinic, 200 First Street SW, Rochester, MN, 55905, USA.

Aims: Right atrial pressure (RAP) provides a composite measure of right ventricular diastolic dysfunction, right atrial compliance, and volume status, and these three variables are typically abnormal in adults with repaired tetralogy of Fallot (TOF). RAP is a well-established prognostic metric in patients with pulmonary hypertension, and recent data suggest that RAP is associated with clinical outcomes in TOF. The purpose of this study was to determine the role of inferior vena cava (IVC) haemodynamics (size and collapsibility) for the assessment of RAP and its potential application for risk stratification and prognostication in the TOF population.

Methods And Results: Adult TOF patients with echocardiographic assessment of IVC haemodynamics were divided into patients with (derivation cohort, n = 256) and without (validation cohort, n = 492) cardiac catheterization data. We assessed the correlation between IVC haemodynamics, RAP, and disease severity indices [arrhythmias, peak oxygen consumption (VO ), and heart failure hospitalization] in derivation cohort and compared it with the correlations in the validation cohort. IVC haemodynamics correlated with RAP (r = 0.52, P < 0.001), with disease severity indices {atrial arrhythmias [area under the curve (AUC) 0.81], ventricular arrhythmias [AUC 0.67], heart failure hospitalizations [AUC 0.78], and peak VO [r = 0.53]}, and with transplant-free survival in the derivation cohort. Similar correlations between IVC haemodynamics, disease severity indices, and transplant-free survival were also observed in the validation cohort.

Conclusions: These findings suggest that IVC haemodynamics can potentially be used for risk stratification and prognostication in TOF patients and can complement the current risk models that are based predominately on right ventricular volumes and systolic function.
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http://dx.doi.org/10.1002/ehf2.12836DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7524124PMC
October 2020

Increased prevalence of left ventricular diastolic dysfunction in adults with repaired coarctation of aorta.

Int J Cardiol Heart Vasc 2020 Jun 18;28:100530. Epub 2020 May 18.

The Department of Cardiovascular Medicine, Mayo Clinic, Rochester, MN 55905, United States.

Background: Left ventricular (LV) pressure overload and coronary artery disease are common in patients with coarctation of aorta (COA), and they are risk factors for LV diastolic dysfunction. Patients with COA may have aortic vasculopathy that can result in LV pressure overload even in the absence of hemodynamically significant COA. We therefore hypothesized that patients with mild COA (without hemodynamically significant COA) will have more LV diastolic dysfunction compared to controls.

Methods: Adult patients with mild COA (Doppler peak velocity < 2.5 m/s) were matched 1:1 to patients without structural heart disease using propensity score method based on age, sex, body mass index, hypertension and blood pressure. The objective was to compare LV diastolic dysfunction (defined as E/e' > 2 standard deviations above age-specific normative values) between adults with repaired COA and controls.

Results: Of 204 COA and 204 control patients (age 35 ± 12 years), patients with COA had higher septal and lateral E/e' ratio (12 ± 4 vs 9 ± 4, p = 0.009) and (10 ± 3 vs 7 ± 3, p < 0.001), respectively. Compared to controls, the prevalence of LV diastolic dysfunction was higher in patients with COA for every age group: <40 years (63% vs 13%, p < 0.001); 41-60 years (87% vs 33%, p < 0.001); age > 60 years (82% vs 56%, p = 0.076). Left ventricular mass index (LVMI) was the strongest determinant of E/e' (β = 2.71 per 10 g/m, standard error = 1.25, p < 0.001).

Conclusion: LV diastolic dysfunction was common in patients with COA, and the association with LVMI suggests that patients with COA may have ongoing LV pressure overload in the absence of hemodynamically significant re-coarctation.
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http://dx.doi.org/10.1016/j.ijcha.2020.100530DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7240224PMC
June 2020

Cardiovascular surgery in Turner syndrome - early outcome and long-term follow-up.

World J Cardiol 2020 Mar;12(3):97-106

Department of Cardiovascular Medicine, Mayo Clinic, Rochester, MN 55905, United States.

Background: Cardiovascular disease is the leading cause of death in patients with Turner syndrome (TS), and cardiovascular surgery is frequently required for management of these patients. TS is associated with medical comorbidities than can complicate the care of this patient population.

Aim: To describe the cardiovascular surgical outcomes of patients with TS.

Methods: A retrospective case series was compiled of 51 consecutive TS patients who had at least one cardiovascular surgery at Mayo Clinic Rochester from 1977-2017. The baseline clinical data of these patients were reviewed including demographics, medical comorbidities, congenital heart disease history, and medications. Echocardiographic reports were analyzed in detail. Operative reports and surgical hospital courses were reviewed. Long-term mortality was determined using medical records and the Social Security Death Index. Survival analysis was performed with the Kaplan Meier method.

Results: The cohort comprised 51 TS patients, average age at the time of surgery at Mayo Clinic was 28 (8-41) years, and 23 (45%) patients were under the age of 18. At the time of first Mayo Clinic surgery, 18 (35%) patients had previously undergone cardiac surgery at another institution. The most common procedures were repair of aortic coarctation in 14 (28%) patients, aortic valve replacement in 6 (12%) patients, and composite aortic root/ascending aorta replacement in 7 (14%) patients, with 7 patients undergoing repair of more than one lesion. Aortic dissection required operative intervention in 5 patients. After initial Mayo Clinic surgery, subsequent operations were required in 6 (13%) patients. Average hospital length of stay was 6 ± 2 d. There were 4 (8%) early surgical deaths. Freedom from death was 97% and 89% at 10 and 20 years, and the freedom from reoperation was 93% and 81% at 10 and 20 years.

Conclusion: Cardiovascular surgery is associated with 8% early mortality given the medical complexity of TS patients. Those who survive to dismissal have good survival. Later cardiovascular reoperations are not rare.
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http://dx.doi.org/10.4330/wjc.v12.i3.97DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7138866PMC
March 2020

Prognostic Implications of Left Ventricular Cardiomyopathy in Adults With Tetralogy of Fallot.

CJC Open 2020 Jan 18;2(1):1-7. Epub 2019 Nov 18.

Department of Cardiovascular Medicine, Mayo Clinic, Rochester, Minnesota, USA.

Background: Despite the significant risk of cardiovascular mortality after tetralogy of Fallot (TOF) repair, there are limited data about left ventricular (LV) cardiomyopathy in this population, thus creating important knowledge gaps. This study aims to address some of these knowledge gaps by describing the risk and prognostic implications of LV systolic dysfunction (LVD) after TOF repair.

Methods: We performed a cohort study of adult patients after TOF repair with an echocardiographic assessment of LV ejection fraction (LVEF) to determine the association between LVD and cardiovascular events, defined as sustained ventricular tachycardia, aborted sudden death, heart transplantation, or death. Prevalent and incidence LVD were defined as LVEF < 50% at baseline or new decrease in LVEF to < 50% during follow-up, respectively.

Results: Of 574 patients (age 38 ± 13 years), the baseline LVEF was 57% ± 9% and 68 (12%) had prevalent LVD. Cardiovascular events occurred in 126 patients (22%) during 10.5 ± 6.2 years of follow-up. LVEF was an independent predictor of mortality (hazard ratio, 1.16; 95% confidence interval, 1.16-1.24;  = 0.003) per 5%-point decrease in LVEF. Among the 357 patients with preserved LVEF and echocardiographic follow-up, incident LVD occurred in 23 (6%) during 3.8 ± 1.6 years of follow-up. Event-free survival was significantly lower in patients with incident LVD compared with patients without incident LVD (87% vs 71%,  = 0.021).

Conclusion: Prevalent and incident LVD occurred in 12% and 6% of this cohort, respectively, and were associated with lower event-free survival. Incident LVD suggests the presence of subclinical LV cardiomyopathy, and further studies are required to determine optimal strategies for diagnosing and treating subclinical LV cardiomyopathy to improve outcomes in the population with TOF.
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http://dx.doi.org/10.1016/j.cjco.2019.11.004DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7063625PMC
January 2020

Echocardiographic predictors of severe right ventricular diastolic dysfunction in tetralogy of Fallot: Relations to patient outcomes.

Int J Cardiol 2020 05 27;306:49-55. Epub 2020 Feb 27.

Department of Cardiovascular Medicine, Mayo Clinic Rochester, MN 55905, United States of America.

Background: Previous studies have described echocardiographic indices of right ventricular (RV) diastolic function in patients with tetralogy of Fallot (TOF) but these indices have not been validated against invasive hemodynamic data. The purpose of this study was to determine echocardiographic predictors of severe RV diastolic dysfunction, and the impact of severe RV diastolic dysfunction on transplant-free survival.

Methods: Cohort study of TOF patients that underwent non-simultaneous cardiac catheterization and echocardiogram at Mayo Clinic. Based on prior studies we selected these indices for assessment: tricuspid E/A, E/e', deceleration time, pulmonary artery forward flow, dilated inferior vena cava (IVC), and hepatic vein diastolic flow reversal (HVDFR). RV diastolic function classes (normal, mild/moderate and severe dysfunction) were created using arbitrary cut-off points of the median values of right ventricular end-diastolic pressure (RVEDP) and right atrial pressure (RAP) for the cohort.

Results: Among 173 patients (age 40 ± 13 years), 68 patients were classified as normal (RVEDP≤14 and RAP≤10), 37 as mild/moderate dysfunction (either RVEDP>14 or RAP>10), and 69 as severe dysfunction (RVEDP>14 and RAP>10). Of the indices assessed, dilated IVC had the best sensitivity of 95% (area under the curve [AUC] 0.689) while HVDFR had the best specificity of 69% (AUC 0.648) for detecting severe RV diastolic dysfunction. Severe RV diastolic dysfunction was an independent risk factor for death/transplant (hazard ratio 2.83, p = 0.009).

Conclusion: Severe RV diastolic dysfunction, as defined by invasive hemodynamic indices, was associated with poor prognosis. Echocardiographic indices can identify these high risk patients, and hence improve risk stratification in clinical practice.
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http://dx.doi.org/10.1016/j.ijcard.2020.02.067DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7297267PMC
May 2020

Long-Term Outcomes of Anticoagulation for Bioprosthetic Valve Thrombosis.

J Am Coll Cardiol 2020 03;75(8):857-866

Department of Cardiovascular Medicine, Mayo Clinic, Rochester, Minnesota. Electronic address:

Background: Early in the prevention and treatment of bioprosthetic valve thrombosis (BPVT), anticoagulation is effective, but the long-term outcome after BPVT is unknown.

Objectives: The goal of this study was to assess the long-term outcomes of patients with BPVT treated with anticoagulation.

Methods: This analysis was a matched cohort study of patients treated with warfarin for suspected BPVT at the Mayo Clinic between 1999 and 2017.

Results: A total of 83 patients treated with warfarin for suspected BPVT (age 57 ± 18 years; 45 men [54%]) were matched to 166 control subjects; matching was performed according to age, sex, year of implantation, and prosthesis type and position. Echocardiography normalized in 62 patients (75%) within 3 months (interquartile range [IQR]: 1.5 to 6 months) of anticoagulation; 21 patients (25%) did not respond to warfarin. Median follow-up after diagnosis was 34 months (IQR: 17 to 54 months). There was no difference in the primary composite endpoint between the patients with BPVT and the matched control subjects (log-rank test, p = 0.79), but the former did have a significantly higher rate of major bleeding (12% vs. 2%; p < 0.0001). BPVT recurred (re-BPVT) in 14 (23%) responders after a median of 23 months (IQR: 11 to 39 months); all but one re-BPVT patient responded to anticoagulant therapy. Patients with BPVT had a higher probability of valve re-replacement (68% vs. 24% at 10 years' post-BPVT; log-rank test, p < 0.001).

Conclusions: BPVT was associated with re-BPVT and early prosthetic degeneration in a significant number of patients. Indefinite warfarin anticoagulation should be considered after a confirmed BPVT episode, but this strategy must be balanced against an increased risk of bleeding.
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http://dx.doi.org/10.1016/j.jacc.2019.12.037DOI Listing
March 2020

Determinants of Left Ventricular Diastolic Function and Exertional Symptoms in Adults With Coarctation of Aorta.

Circ Heart Fail 2020 02 14;13(2):e006651. Epub 2020 Feb 14.

Department of Cardiovascular Medicine (A.C.E., H.M.C.), Mayo Clinic, Rochester, MN.

Background: Coarctation of aorta (COA) results in chronic left ventricular (LV) pressure overload and subsequently leads to LV diastolic dysfunction and heart failure over time. The goal of COA intervention is to prevent these complications. The timing of COA interventions is based on the presence of these COA severity indices: doppler mean COA gradient, systolic blood pressure, upper-to-lower-extremity SBP gradient, aortic isthmus ratio, presence of collaterals, and exercise-induced hypertension. Although these indices are physiologically intuitive, the relationship between these indices and LV diastolic dysfunction and exertional symptoms has not been studied. The purpose of this study was to evaluate the association between the indices of COA severity and LV diastolic function and symptoms.

Methods: In this cross-sectional study, multivariate linear and logistic regression analyses were used to assess the correlation between indices of COA severity, LV diastolic function (average e' and E/e'), and exertional symptoms (NYHA II-IV and peak oxygen consumption).

Results: Of all the COA indices analyzed in 546 adult COA patients, aortic isthmus ratio had the strongest correlation with e' (β [95% CI]: 3.11 [2.02-4.31]; =0.014) per 1 cm/second; E/e' (-13.4 [-22.3 to -4.81]; =0.009) per 1 unit; peak oxygen consumption (4.05 [1.97-6.59] per 1% change, =0.019), and NYHA II to IV symptoms (odds ratio, 2.16 [1.65-3.18]; =0.006).

Conclusions: Of all the COA severity indices stipulated in the guidelines, aortic isthmus ratio had the strongest correlation with LV diastolic function and exertional symptoms. As LV diastolic dysfunction typically precede heart failure symptoms, we anticipate that the results of this study will improve and simplify patient selection for COA intervention and potentially improve long-term outcomes.
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http://dx.doi.org/10.1161/CIRCHEARTFAILURE.119.006651DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7041882PMC
February 2020

Incidence and outcomes of prosthetic valve endocarditis in adults with tetralogy of Fallot.

Cardiol Young 2020 Jan 8;30(1):19-23. Epub 2020 Jan 8.

Department of Cardiovascular Medicine, Mayo Clinic Rochester, Rochester, MN, USA.

Background: The risk of endocarditis varies with CHD complexity and the presence of prosthetic valves. The purpose of the study was therefore to describe incidence and outcomes of prosthetic valve endocarditis in adults with repair tetralogy of Fallot.

Methods: Retrospective review of adult tetralogy of Fallot patients who underwent prosthetic valve implantation, 1990-2017. We defined prosthetic valve endocarditis-related complications as prosthetic valve dysfunction, perivalvular extension of infection such abscess/aneurysm/fistula, heart block, pulmonary/systemic embolic events, recurrent endocarditis, and death due to sepsis.

Results: A total of 338 patients (age: 37 ± 15 years) received 352 prosthetic valves (pulmonary [n = 308, 88%], tricuspid [n = 13, 4%], mitral [n = 9, 3%], and aortic position [n = 22, 6%]). The annual incidence of prosthetic valve endocarditis was 0.4%. There were 12 prosthetic valve endocarditis-related complications in six patients, and these complications were prosthetic valve dysfunction (n = 4), systemic/pulmonary embolic events (n = 2), heart block (n = 1), aortic root abscess (n = 1), recurrent endocarditis (n = 2), and death due to sepsis (n = 1). Three (50%) patients required surgery at 2 days, 6 weeks, and 23 weeks from the time of prosthetic valve endocarditis diagnosis. Altogether three of the six (50%) patients died, and one of these deaths was due to sepsis.

Conclusions: The incidence, complication rate, and outcomes of prosthetic valve endocarditis in tetralogy of Fallot patients underscore some of the risks of having a prosthetic valve. It is important to educate the patients on the need for early presentation if they develop systemic symptoms, have a high index of suspicion for prosthetic valve endocarditis, and adopt a multi-disciplinary care approach in this high-risk population.
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http://dx.doi.org/10.1017/S1047951119001975DOI Listing
January 2020

Left Ventricular Systolic Dysfunction and Cardiovascular Outcomes in Tetralogy of Fallot: Systematic Review and Meta-analysis.

Can J Cardiol 2019 12 8;35(12):1784-1790. Epub 2019 Aug 8.

Department of Cardiovascular Medicine Mayo Clinic, Rochester, Minnesota, USA.

Background: Although there are robust data about the pathophysiology and prognostic implications of left ventricular (LV) systolic dysfunction in patients with acquired heart disease, similar prognostic data about LV systolic dysfunction are sparse in the tetralogy of Fallot (TOF) population. The purpose of this study was to perform a meta-analysis of all studies that assessed the relationship between LV ejection fraction (LVEF) and cardiovascular adverse events (CAEs) defined as death, aborted sudden death, or sustained ventricular tachycardia.

Methods: We used random-effects models to calculate hazard ratios (HRs) and 95% confidence intervals (CIs).

Results: Of the 1,809 citations, 7 studies with 2,854 patients (age 28 ± 4 years) were included. During 5.6 ± 3.4 years' follow-up, there were 82 deaths, 17 aborted sudden cardiac deaths, and 56 sustained ventricular tachycardia events. Overall, CAEs occurred in 5.1% (144 patients). As a continuous variable, LVEF was a predictor of CAE (HR 1.29, 95% CI, 1.09-1.53, P = 0.001) per 5% decrease in LVEF. Similarly, LVEF < 40% was also a predictor of CAE (HR 3.22, 95% CI, 2.16-4.80, P < 0.001).

Conclusions: LV systolic dysfunction was an independent predictor of CAE, and we observed a 30% increase in the risk of CAE for every 5% decrease in LVEF, and a 3-fold increase in the risk of CAE in patients with LVEF <40% compared with other patients. These findings underscore the importance of incorporating LV systolic function in clinical risk stratification of patients with TOF and the need to explore new treatment options to address this problem.
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http://dx.doi.org/10.1016/j.cjca.2019.07.634DOI Listing
December 2019

Suprasternal Pulmonary Artery Puncture in the Setting of Right-Sided Mechanical Prostheses: The Radner Technique Redux.

JACC Cardiovasc Interv 2019 11 30;12(22):2320-2321. Epub 2019 Oct 30.

Department of Pediatric and Adolescent Medicine/Division of Pediatric Cardiology, Mayo Clinic, Rochester, Minnesota.

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http://dx.doi.org/10.1016/j.jcin.2019.05.036DOI Listing
November 2019

Impact of atrial arrhythmia on survival in adults with tetralogy of Fallot.

Am Heart J 2019 12 20;218:1-7. Epub 2019 Aug 20.

Department of Cardiovascular Medicine, Mayo Clinic Rochester, MN.

Background: Atrial arrhythmia is a late complication after tetralogy of Fallot (TOF) repair, but arrhythmia outcomes data are limited.

Objectives: The purpose of the study was to describe atrial arrhythmia presentations, outcomes of antiarrhythmic therapy, and impact of arrhythmia on transplant-free survival.

Methods: We reviewed the MACHD (Mayo Adult Congenital Heart Disease) Registry and identified 113 patients (age 49 ± 13 years) with documented arrhythmia, and 302 patients without history of arrhythmia, 1990-2017. We classified arrhythmias into atrial fibrillation and atrial flutter/tachycardia based on the rhythm on the first abnormal electrocardiogram.

Results: At the time of first documented arrhythmia, 58(51%) had atrial fibrillation while 55(49%) had atrial flutter/tachycardia. Of the 113 patients, 14(12%) received rhythm control with class I/III antiarrhythmic drugs (AAD), 79(70%) had direct current cardioversion, 9(8%) received rate control with class II/IV AAD, and 11(10%) received only anticoagulation. Successful cardioversion occurred in 100(89%) patients, and arrhythmia recurrence rate was 16 per 100 patient-years. The multivariate risk factors for death and/or heart transplant were atrial fibrillation (HR 1.94, CI 1.10-3.15, P = .031) and older age (HR 1.63, CI 1.12-2.43, P = .019) per 5 year increment.

Conclusions: Atrial fibrillation, but not atrial flutter, was associated with reduced survival in our repaired TOF cohort. Further studies are required to determine if more aggressive antiarrhythmic therapy will improve survival in patients with atrial fibrillation.
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http://dx.doi.org/10.1016/j.ahj.2019.08.013DOI Listing
December 2019

In Reply.

Obstet Gynecol 2019 10;134(4):882

University of Mississippi Medical Center, Jackson, Mississippi.

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http://dx.doi.org/10.1097/AOG.0000000000003498DOI Listing
October 2019

Cerebrovascular accidents in Ebstein's anomaly.

Congenit Heart Dis 2019 Nov 23;14(6):1157-1165. Epub 2019 Sep 23.

Department of Cardiovascular Diseases, Mayo Clinic, Rochester, Minnesota.

Introduction: Mechanisms and risk factors for cerebrovascular accidents (CVAs) in Ebstein's anomaly (EA) are not well understood; hence, we aimed to clarify these in a large cohort of EA patients.

Methods: Patients with a confirmed diagnosis of EA were retrospectively reviewed. Baseline characteristics were compared between patients with and without a prior history of CVA using logistic regression modeling. Cox regression analysis was used to identify predictors of CVA following initial evaluation. CVA incidence from birth and following tricuspid valve surgery were estimated using the Kaplan-Meier method.

Results: Nine hundred sixty-eight patients (median age 21.1 years, 41.5% male) were included, in which, 87 patients (9.0%) had a history of CVA (54 strokes, 33 transient ischemic attacks; 5 associated with brain abscesses) prior to their initial evaluation. The odds of atrial septal defect/patent foramen ovale (odds ratio [OR] 4.91; 95% CI 2.60-21.22; p = .0002) and migraines/headaches (OR 2.38; 95% CI 1.40-4.04; p = .0013) but not atrial arrhythmias (OR 0.75; 95% CI 0.44-1.30; p = .31) were significantly higher among patients with prior CVA following multivariable adjustment. Seventeen patients experienced CVA following initial evaluation; no examined variables including atrial arrhythmias (HR 2.38; 0.91-6.19; p = .076) were predictive of CVA risk. The 10-year, 50-year, and 70-year incidences of CVA were 1.4%, 15.9%, and 23.5%, respectively, with paradoxical embolism heavily implicated.

Conclusion: Patients with EA are at substantive risk for CVA. Histories of migraines/headaches and interatrial shunts should prompt concern for paradoxical embolic CVAs. This has significant implications for all patients with atrial-level shunting.
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http://dx.doi.org/10.1111/chd.12841DOI Listing
November 2019

Quality of Life of Patients With Marfan Syndrome After Valve-Sparing or Valve-Replacement Operations.

Mayo Clin Proc 2019 09;94(9):1906-1909

Department of Cardiovascular Medicine, Mayo Clinic, Rochester, MN.

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http://dx.doi.org/10.1016/j.mayocp.2019.04.045DOI Listing
September 2019

Usefulness of Right Ventricular Volumetric and Afterload Indices for Risk Stratification in Patients With Tetralogy of Fallot.

Am J Cardiol 2019 10 28;124(8):1293-1297. Epub 2019 Jul 28.

Department of Cardiovascular Medicine, Mayo Clinic Rochester, Minnesota.

Right ventricular (RV) volume overload due to chronic pulmonary regurgitation is the common mechanism for hemodynamic deterioration after tetralogy of Fallot (TOF) repair. As a result, RV volumetric indices are used for clinical risk stratification in this population. Since RV afterload is a determinant of RV hemodynamic performance, we hypothesized that afterload-adjusted RV volumetric indices will have a better correlation with disease severity compared with RV volumetric indices alone in patients with TOF. Cross-sectional study of adults with previous TOF repair that received care at Mayo Clinic, 2002-2015. We defined disease severity as atrial arrhythmia and/or impaired exercise capacity. We created afterload-adjusted RV volumetric indices by indexing these indices to RV systolic pressure (RVSP) as follows: RV end-diastolic volume (RVEDVi)/RVSP, RV end-systolic volume (RVESVi)/RVSP, and RV ejection fraction (RVEF)/RVSP. The RV volumetric indices were: RVEDVi 141 ± 43 ml/m, RVESVi 79 ± 38 ml/m, and RVEF 44 ± 10%, and RVSP was 48 ± 9 mm Hg. RVESVi was the only RV volumetric parameter that was associated with disease severity (odds ratio [OR] 1.13, 95% confidence interval [CI] 1.01 to 1.32, p = 0.041) with area under the curve (AUC) of 0.612. In contrast RVEF/RVSP (OR 0.73, 95% CI 0.38 to 0.92, p = 0.037, AUC 0.649), and RVESVi/RVSP (OR 1.28, 95% CI 1.14-1.55, p = 0.008, AUC 0.798) were associated with disease severity. Compared with RV volumetric indices alone, the combined RV volumetric and afterload indices had better correlation with disease severity as measured by AUC. Afterload-adjusted RV volumetric indices had better correlation with disease severity, and may potentially improve risk stratification in this population.
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http://dx.doi.org/10.1016/j.amjcard.2019.07.025DOI Listing
October 2019

Echo-Doppler Assessment of Left Filling Pressures in Adults With Repaired Tetralogy of Fallot.

Circ Cardiovasc Imaging 2019 08 15;12(8):e009195. Epub 2019 Aug 15.

Department of Cardiovascular Medicine (W.R.M., H.M.C., J.K.O., A.C.E.), Mayo Clinic, MN.

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http://dx.doi.org/10.1161/CIRCIMAGING.119.009195DOI Listing
August 2019

Ebstein's Anomaly.

Methodist Debakey Cardiovasc J 2019 Apr-Jun;15(2):138-144

MAYO CLINIC, ROCHESTER, MINNESOTA.

Ebstein's anomaly is a malformation of the tricuspid valve with myopathy of the right ventricle (RV) that presents with variable anatomic and pathophysiologic characteristics, leading to equally variable clinical scenarios. Medical management and observation is often recommended for asymptomatic patients and may be successful for many years. Tricuspid valve repair is the goal of operative intervention; repair also typically includes RV plication, right atrial reduction, and atrial septal closure or subtotal closure. Postoperative functional assessments generally demonstrate an improvement or relative stability related to degree of RV enlargement, RV dysfunction, RV fractional area change, and tricuspid valve regurgitation.
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http://dx.doi.org/10.14797/mdcj-15-2-138DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6668741PMC
August 2019