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Ann Thorac Surg 2020 Jun 30. Epub 2020 Jun 30.

Lund University, Skane University Hospital, Department of Clinical Sciences Lund, Pediatric Cardiology, Lund, Sweden.

Background: Following surgical repair of aortic coarctation (CoA) there is a risk for restenosis (reCoA), particularly in the first year of life. It was the aim of this study to identify reCoA risk factors by analyzing postoperative pre-discharge echocardiograms.

Methods: Retrospective analysis of echocardiograms of children born operated for CoA in Sweden 2011-2017. Read More

Eur Heart J Case Rep 2020 Jun 15;4(3):1-5. Epub 2020 May 15.

Department of Cardiology, University and Hospital of Fribourg, Chemin des Pensionnats 2, 1708 Fribourg, Switzerland.

Background: Aortic pseudocoarctation (PsCoA) is an elongation of the supra-isthmic aorta with kinking and low-grade narrowing. Consequently, no collateral circulation is found. It is frequently associated with other congenital heart anomalies, such as bicuspid aortic valve (BAV). Read More

Eur Heart J Case Rep 2020 Jun 24;4(3):1-4. Epub 2020 Mar 24.

Department of Cardiothoracic Surgery, Barts Heart Centre, St Bartholomew's Hospital, West Smithfield, London EC1A 7BE, UK.

Background: Klippel-Feil syndrome (KFS) is a rare congenital anomaly of the cervical spine, which is associated with a number of cardiovascular malformations, including coarctation of the aorta, bicuspid aortic valve (BAoV), and aortic aneurysm. Operative management of aortic stenosis of a BAoV in a patient with KFS has not been previously reported.

Case Summary: A 54-year-old Caucasian woman with known KFS presented to her local hospital for elective cholecystectomy. Read More

Eur Heart J Case Rep 2020 Jun 3;4(3):1-5. Epub 2020 May 3.

Department of Cardiology, University Medical Center Utrecht, Heidelberglaan 100, 3584 CX Utrecht, The Netherlands.

Background: Aortitis is a rare condition that can be caused by inflammatory or infectious aetiologies. The clinical presentation of aortitis includes a heterogeneous range of symptoms and clinical signs.

Case Summary: We present a 53-year-old man whose medical history included presence of a ventricular septal defect, a bicuspid aortic valve, and coarctation of the aorta. Read More

J Ultrason 2020 15;20(81):e146-e150. Epub 2020 Jun 15.

Department for Prenatal Cardiology, Polish Mother's Memorial Hospital Research Institute , Lodz , Poland ; Department for Congenital Malformations and Prevention, Faculty of Public Health, Medical University of Lodz , Poland.

Persistent left superior vena cava is a malformation of cardinal veins. We report a case of a secundigravida who had many fetal ultrasound examinations - first performed by an obstetrician (who described fetal mediastinum as "abnormal"), second by other obstetrician, who performed basic fetal echocardiographic examination and diagnosed persistent left superior vena cava. The woman was referred to a tertiary center for detailed fetal echocardiography. Read More

Tex Heart Inst J 2020 Apr;47(2):149-151

Division of Cardiothoracic Surgery, Michael E. DeBakey Department of Surgery, Baylor College of Medicine; and Department of Cardiovascular Surgery, Texas Heart Institute; Houston, Texas 77030.

Successful surgical repair of aortic coarctation during childhood may have major late complications such as pseudoaneurysm formation. If left untreated, pseudoaneuryms put patients at risk for morbidity and death; if treated surgically, they are associated with complications. Endovascular aortic repair, an established safe alternative to open surgical repair, is associated with encouraging outcomes and fewer complications, and it is especially feasible for patients who have undergone multiple aortic surgeries. Read More

BMC Pediatr 2020 Jun 29;20(1):320. Epub 2020 Jun 29.

Department of Neonatology, Neonatal Biophysical Monitoring and Cardiopulmonary Therapies Research Unit, Poznan University of Medical Sciences, Poznan, Poland.

Background: Alveolar capillary dysplasia (ACD) is a rare cause of severe pulmonary hypertension and respiratory failure in neonates. The onset of ACD is usually preceded by a short asymptomatic period. The condition is refractory to all available therapies as it irreversibly affects development of the capillary bed in the lungs. Read More

Evid Based Complement Alternat Med 2020 9;2020:2957094. Epub 2020 Jun 9.

Department of Nursing, Xinjiang Medical University, Urumqi 830011, China.

Phenylethanol glycosides (CPhGs) are the core material basis of pharmacological activity in and have a variety of pharmacological effects. However, it is unclear whether CPhGs have an ameliorative effect on pressure overload-induced myocardial hypertrophy. In this study, male SD rats weighing (200 ± 20) g were established cardiac hypertrophy models by abdominal aortic coarctation (AAC). Read More

J Cardiothorac Vasc Anesth 2020 May 27. Epub 2020 May 27.

Department of Anesthesiology, Children's Hospital Colorado, University of Colorado School of Medicine, Aurora, CO. Electronic address:

Objective: Paradoxical hypertension after repair of coarctation of the aorta in children is associated with the release of catecholamines and activation of the renin-angiotensin system. The objective of the present study was to describe the effects of labetalol infusion on blood pressure, plasma catecholamine levels, and plasma renin activity in a series of children undergoing repair of coarctation of the aorta.

Design: Prospective, observational cohort study. Read More

Am J Cardiol 2020 May 26. Epub 2020 May 26.

Department of Pediatric Cardiology, National Cerebral and Cardiovascular Center, Suita, Osaka, Japan.

Several studies have reported aortic dilation and increased stiffness of the ascending aorta in patients after repair of congenital heart disease (CHD), which may be a predominant cardiovascular risk. However, the clinical significance has not been described in detail. In this retrospective study, 175 repaired patients with complex CHD achieving biventricular circulation and age-matched 39 control subjects were reviewed (median age: 14. Read More

ESC Heart Fail 2020 Jun 25. Epub 2020 Jun 25.

Department of Cardiology, AZ Nikolaas General Hospital, Moerlandstraat 1, Sint-Niklaas, B-9100, Belgium.

Patients with adult congenital heart disease are born with structural heart defects who survived into adulthood. Occasionally, complex lesions remain undiagnosed, potentially causing substantial cardiovascular health problems at young age. Here, the case is presented of a patient with subacute heart failure 1 week postpartum, revealing the diagnosis of aortic coarctation (CoA) with patent ductus arteriosus (PDA). Read More

J Cell Mol Med 2020 Jun 24. Epub 2020 Jun 24.

Institute of Cardiology, The First Affiliated Hospital, College of Medicine, Zhejiang University, Hangzhou, China.

Farnesyltransferase (FTase) is an important enzyme that catalyses the modification of protein isoprene downstream of the mevalonate pathway. Previous studies have shown that the tissue of the heart in the suprarenal abdominal aortic coarctation (AAC) group showed overexpression of FTaseβ (FNTB) and the activation of the downstream protein Ras was enhanced. FTase inhibitor (FTI) can alleviate myocardial fibrosis and partly improve cardiac remodelling in spontaneously hypertensive rats. Read More

CASE (Phila) 2020 Jun 27;4(3):142-145. Epub 2020 Mar 27.

Division of Cardiology, Rady Children's Hospital San Diego, San Diego, California.

Clin Case Rep 2020 Jun 10;8(6):1021-1024. Epub 2020 Mar 10.

Cardiovascular Research Center Shahid Beheshti University of Medical Sciences Tehran Iran.

Double orifice mitral valve is a rare condition and may be accompanied by a bicuspid aortic valve (sometimes normal functioning) and coarctation of the aorta. Echocardiography is valuable in detecting the accompanied anomalies. Management depends on the severity of mitral valve function and the severity of associated anomalies. Read More

ESC Heart Fail 2020 Jun 19. Epub 2020 Jun 19.

Department of Cardiology, Faculty of Medicine, University of Tsukuba, 1-1-1, Tennodai, Tsukuba, Ibaraki, 305-8575, Japan.

Atypical aortic coarctation is a rare condition associated with Takayasu arteritis, and is characterized by symptoms caused either by hypotension in the lower half of the body or secondary hypertension in the upper half of the body, and heart failure. We report a rare case of axillofemoral bypass to improve congestive heart failure for atypical aortic coarctation complicating Takayasu arteritis. Augmented vascular bed and retrograde renal blood flow after axillofemoral bypass surgery could achieve effective blood pressure control and improve renal function and cardiac function (LVEF: 30% → 55%, BNP: 2943 pg/mL → 128 pg/mL). Read More

J Card Surg 2020 Jun 19. Epub 2020 Jun 19.

Division of Thoracic and Cardiovascular Surgery, College of Medicine, University of Florida, Gainesville, Florida.

Objective: This study highlights the management strategy in simultaneous bicuspid aortic valve infective endocarditis and mycotic pseudoaneurysm of an aortic coarctation.

Methods: A staged repair of mycotic pseudoaneurysm of aortic coarctation and infective bicuspid aortic valve endocarditis.

Results: We present a 19-year old gentleman who was admitted with aching pain in his bilateral lower extremities with associated purpuric rash and fevers. Read More

J Inherit Metab Dis 2020 Jun 18. Epub 2020 Jun 18.

Service de Génétique, CHU-Hôpital Nord, Saint-Etienne, France.

5-amino-4-imidazolecarboxamide-ribosiduria (AICA)-ribosiduria is an exceedingly rare autosomal recessive condition resulting from the disruption of the bifunctional purine biosynthesis protein PURH (ATIC), which catalyzes the last two steps of de novo purine synthesis. It is characterized biochemically by the accumulation of AICA-riboside in urine. AICA-ribosiduria had been reported in only one individual, 15 years ago. Read More

Braz J Cardiovasc Surg 2020 Jun 1;35(3):254-264. Epub 2020 Jun 1.

Universidade de São Paulo Faculdade de Medicina de Ribeirão Preto Department of Internal Medicine Ribeirão Preto SP Brazil Cardiology Center of the Department of Internal Medicine, Faculdade de Medicina de Ribeirão Preto da Universidade de São Paulo (FMRP-USP), Ribeirão Preto, SP, Brazil.

Objective: Description of adult congenital heart disease (CHD) outpatient characteristics has not been reported and several aspects regarding these patients require attention. We describe the 12-year experience of a Brazilian unit.

Methods: The main characteristics of 1168 patients were reviewed annotating for each patient age, gender, city of residence, main diagnosis, functional class at last examination, defect complexity and in-hospital referral pattern. Read More

J Cardiol Cases 2020 Jun 17;21(6):238-241. Epub 2020 Mar 17.

Department of Medical Science and Cardiorenal Medicine, Yokohama City University Graduate School of Medicine, 3-9, Fukuura, Kanazawa, Yokohama 236-0004, Japan.

The patient was a 19-year-old woman who had experienced headache for 1 year. Soon after birth, ventricular septal defects were diagnosed, the size of which were small, therefore not requiring surgical repair. She also noticed hypertension, with up to 184/110 mmHg of blood pressure. Read More

J Cardiovasc Comput Tomogr 2020 May 16. Epub 2020 May 16.

Department of Cardiology, All India Institute of Medical Sciences, New Delhi, 110029, India.

Acta Cardiol 2020 Jun 15:1-2. Epub 2020 Jun 15.

Department of Cardiovascular Radiology and Endovascular Interventions, All India Institute of Medical Sciences, New Delhi, India.

J Cardiothorac Surg 2020 Jun 11;15(1):136. Epub 2020 Jun 11.

Heart Center, Qingdao Women and Children's Hospital, Qingdao University, 6 tongfu Road, Qingdao, Shandong, China.

Transposition of the great arteries (TGA) and interruption of the aortic arch (IAA) are uncommon congenital heart diseases. The association between TGA and IAA is rare. The aim of this study is to present a case with combined TGA and IAA, who underwent the primary repair and review the literature with similar cases. Read More

Cureus 2020 Jun 5;12(6):e8453. Epub 2020 Jun 5.

Radiology, King Abdulaziz Medical City, Ministry of National Guard Health Affairs, Jeddah, SAU.

In this report, we describe the case of a 13-year-old male who presented to our hospital after sustaining injuries from a motor vehicle accident (MVA). A full-body CT scan revealed multiple injuries, including subgaleal hematoma, hemorrhagic brain contusion, moderate-size aortic pseudoaneurysm with associated mediastinal hematoma, and multiple stable visceral organ lacerations. Additionally, there were numerous fractures, including in the right femur associated with large hematoma. Read More

Cardiol Young 2020 Jun 9:1-4. Epub 2020 Jun 9.

Pediatric Cardiology, Department of Women's and Children's Health, University of Padua, Padova, Italy.

Percutaneous treatment of aortic coarctation is based on angioplasty and/or stenting of the isthmus. We report a case of a 28-year-old girl suffering from aortic coarctation syndrome (coarctation + ventricular septal defect + bicuspid aorta). She underwent coarctectomy with subclavian flap and pulmonary bandage followed by ventricular septal defect closure and bandage removal in her first year of life. Read More

Biomed Res Int 2020 14;2020:3805385. Epub 2020 May 14.

Department of Cardiovascular Surgery, The Second Xiangya Hospital of Central South University, Middle Renmin Road 139, Changsha 410000, China.

Objectives: To evaluate the early and midterm results of a modified sliding anastomosis technique in patients with aortic coarctation.

Materials And Methods: In this study, we reported a new repair method and compared the early and midterm outcome(s) with a conventional surgical approach for the management of patients with aortic coarctation. Forty-eight aortic coarctation patients with a narrowed segment length longer than 2 cm were operated at our department's pediatric surgical division. Read More

Prog Cardiovasc Dis 2020 Jun 1. Epub 2020 Jun 1.

Department of Pediatric and Adolescent Medicine, Division of Pediatric Cardiology, Mayo Clinic, Rochester, MN, United States of America; Department of Cardiovascular Medicine, Mayo Clinic, Rochester, MN, United States of America. Electronic address:

Bicuspid aortic valve (BAV) is one of the most common congenital heart defects in children, adolescents and adults. BAV can occur as an isolated lesion or in association with other congenital heart defects, such as coarctation of the aorta or genetic syndromes like Turner syndrome. Although the majority of long term complications associated with BAV manifest later in life, children and adolescents may present with early onset valvular dysfunction or dilation of the aorta. Read More

Indian J Radiol Imaging 2020 Jan-Mar;30(1):81-83. Epub 2020 Mar 30.

Department of Radiology, IRCCS Policlinico San Matteo Foundation, Pavia, Italy.

Interrupted aortic arch diagnosed in adult age is a rare entity, with only a few cases published in the literature. Most of them are classified as type A interrupted aortic arch and differential diagnosis is associated with severe chronic coarctation. We present a case of a 52-year-old woman accessed to the emergency department for chest and right upper limb pain that increased in the last days. Read More

Am Heart J 2020 Jul 13;225:78-87. Epub 2020 May 13.

Department of Cardiology, University Medical Center Utrecht, Utrecht, the Netherlands.

Background: The severity of aortic coarctation (CoA) may be underestimated during cardiac catheterization. We aimed to investigate whether epinephrine stress testing improves clinical decision making and outcome in CoA.

Methods: We retrospectively evaluated CoA patients >50 kg with a peak systolic gradient (PSG) ≤20 mm Hg during cardiac catheterization who underwent epinephrine stress testing. Read More

Curr Pharm Des 2020 May 31. Epub 2020 May 31.

Unit of Reproductive Endocrinology, 1st Department of Obstetrics and Gynaecology, Medical School, Aristotle University of Thessaloniki, Thessaloniki. Greece.

Turner's or Turner syndrome (TS) is the most prevalent chromosomal abnormality in female live births. Patients with TS are predisposed to increased risk of cardiovascular disease (CVD), mainly due to the frequently observed congenital structural cardiovascular defects, such as valvular and aortic abnormalities (coarctation, dilatation and dissection). The increased prevalence of cardiometabolic risk factors, such as arterial hypertension, insulin resistance, diabetes mellitus, dyslipidaemia, central obesity and increased carotid intima-media thickness, also contribute to increased morbidity and mortality in TS patients. Read More

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. Read More

Folia Morphol (Warsz) 2020 May 27. Epub 2020 May 27.

Background: In this study, we aimed to image pulmonary venous return anomalies and associated cardiovascular and pulmonary abnormalities by high-pitch low-dose computed tomography (CT) in children.

Materials And Methods: Forty-one patients with total or partial anomalous pulmonary venous return anomalous between May 2012 and June 2019 were retrospectively reviewed. The anomalies were determined using high-pitch low-dose CT. Read More

Cureus 2020 May 19;12(5):e8204. Epub 2020 May 19.

Cardiology, King Faisal Cardiac Center, King Abdulaziz Medical City, Ministry of National Guard Health Affairs, Jeddah, SAU.

We describe a case of a 17-year-old male patient who was admitted to the hospital for an evaluation of his recurrent postprandial abdominal pain and fatigue on exertion. He was discovered to have severe post-ductal aortic coarctation (CoA) and uninterrupted left-sided inferior vena cava (IVC) draining into the right atrium crossing anterior to the abdominal aorta. There were no signs of IVC compression. Read More

Philos Trans A Math Phys Eng Sci 2020 Jun 25;378(2173):20190342. Epub 2020 May 25.

Gottfried Schatz Research Center for Cell Signaling, Metabolism and Aging - Division of Biophysics, Medical University Graz, Graz, Austria.

Computer models of left ventricular (LV) electro-mechanics (EM) show promise as a tool for assessing the impact of increased afterload upon LV performance. However, the identification of unique afterload model parameters and the personalization of EM LV models remains challenging due to significant clinical input uncertainties. Here, we personalized a virtual cohort of  = 17 EM LV models under pressure overload conditions. Read More

Eur J Cardiothorac Surg 2020 May 23. Epub 2020 May 23.

Department of Cardiac Surgery, Royal Children's Hospital, Melbourne, VIC, Australia.

Objectives: Management of patients with left ventricular inflow and outflow stenotic lesions can be challenging. Our purpose was to characterize such patients and review the long-term outcomes of those requiring mitral valve (MV) surgery.

Methods: We performed a retrospective study of 40 patients with subaortic, aortic and/or arch stenotic lesion(s) who underwent MV surgery between 1985 and 2016. Read More

Semin Thorac Cardiovasc Surg 2020 May 22. Epub 2020 May 22.

Department of Pediatric Cardiac Surgery, Meshalkin National Medical Research Center, Novosibirsk, Russia.

We aimed to compare the safety and efficacy of 3 perfusion methods primarily used in aortic arch reconstruction in infants, namely, deep hypothermic circulatory arrest, selective antegrade cerebral perfusion, and double arterial cannulation. Forty-five infants with aortic arch obstruction and biventricular anatomy were enrolled in this pilot prospective study (ClinicalTrials.gov registration number: NCT02835703). Read More

Korean J Anesthesiol 2020 May 20. Epub 2020 May 20.

Department of Anesthesia, New Cross Hospital, Wolverhampton Road, Wolverhampton, West Midlands, WV10 0QP, United Kingdom.

Background: Shone's syndrome is a rare complex congenital cardiac condition, characterised by a supra-valvular mitral ring, parachute deformity of the mitral valve, aortic stenosis and coarctation of the aorta.

Case: A 24-year-old parturient with partial Shone's syndrome presented to our delivery unit in pulmonary oedema. She underwent a scheduled cesarean section performed under a combined spinal epidural anesthetic at 33 weeks. Read More

Orv Hetil 2020 05;161(21):861-866

Általános Orvostudományi Kar, Szent-Györgyi Albert Klinikai Központ, II. Belgyógyászati Klinika, Szívsebészeti Osztály,Szegedi Tudományegyetem Szeged, Semmelweis u. 8., 6720.

The surgical management of congenital heart defects is changing continuously due to technical development. Aortic coarctation is one of the most frequent congenital heart diseases often with serious symptoms. The authors review the different surgical techniques (end-to-end anastomosis, pathplasty, interposition) and present the up-to-date procedures from infancy to adulthood. Read More

J Biomech 2020 May 21;105:109796. Epub 2020 Apr 21.

Department of Electronics, Information and Bioengineering, Politecnico di Milano, Milano, Italy.

Endovascular stenting has recently become a standard treatment for native coarctation of the aorta (CoA) in children and young adults, given the efficacy in relieving vessel obstruction with a low incidence of adverse events. Yet, despite the short-term success of the technique, late hypertension remains an endemic risk. To assess the impact of the percutaneous procedure on the aortic wall biomechanics, we designed a novel finite element (FE) protocol for the simulation of endovascular stenting in three patient-specific CoA anatomies, developing a remeshing procedure that allows for coping with different CoA severities. Read More

Comput Methods Programs Biomed 2020 May 11;194:105522. Epub 2020 May 11.

Department of Vascular Surgery, Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, 100037, China. Electronic address:

Background And Objective: Post-Stenotic Dilatation (PSD), the common complication of coarctation of the aorta (COA), is a progressive disease involving aortic aneurysm and even rupture. However, there has been no definitive method that could investigate the mechanism of PSD formation, progression and rupture. The purpose of the present work is to analyze the mechanism behind PSD formation and to further assess the risk of COA patients with different coarctation degrees deteriorating into PSD. Read More

Biomed Res Int 2020 11;2020:7256862. Epub 2020 Jan 11.

Department of Cardiology, Nanjing First Hospital, Nanjing Medical University, No. 68, Changle Road, Nanjing 210006, Jiangsu, China.

Cardiac hypertrophy and ventricular remodeling following heart failure are important causes of high mortality in heart disease patients. The cardiac lymphatic system has been associated with limited research, but it plays an important role in the improvement of myocardial edema and the promotion of fluid balance. LCZ696 is a novel combination of angiotensin and neprilysin inhibitors. Read More

PLoS One 2020 15;15(5):e0228478. Epub 2020 May 15.

Department of Anatomy & Embryology, Leiden University Medical Center, Leiden, The Netherlands.

Objectives: In congenital heart malformations with pulmonary stenosis to atresia an abnormal lateral ductus arteriosus to left pulmonary artery connection can lead to a localised narrowing (pulmonary ductal coarctation) or even interruption We investigated embryonic remodelling and pathogenesis of this area.

Material And Methods: Normal development was studied in WntCre reporter mice (E10.0-12. Read More

J Cardiothorac Surg 2020 May 12;15(1):87. Epub 2020 May 12.

Department of Radiology, Sichuan Academy of Medical Sciences and Sichuan Provincial People's Hospital, Qingyang District, Chengdu, 610072, Sichuan, China.

Background: Pulmonary artery aneurysm (PAA), usually associated with congenital heart disease (CHD), is a very rare clinical condition. Pulmonary artery dissection (PAD) is considered the most life-threatening complication of PAA, and patients can progress from being asymptomatic to sudden death. We report the following case of PAA associated with complicated congenital heart disease and simultaneous chronic PAD. Read More

BMC Cardiovasc Disord 2020 May 11;20(1):216. Epub 2020 May 11.

Department of Cardiovascular Surgery, IMS Tokyo Katsushika General Hospital, 4-18-1, Nishishinkoiwa, Katsushika-ku, Tokyo, 124-0025, Japan.

Background: Jehovah's Witnesses refuse allogeneic blood transfusions, which makes cardiovascular surgery challenging. Surgeons must minimize blood and fluid loss within one procedure.

Case Presentation: We herein describe a 17-year-old male Jehovah's Witness with bicuspid aortic valve regurgitation and coarctation of the aorta. Read More

J Cardiovasc Surg (Torino) 2020 Apr 29. Epub 2020 Apr 29.

Department of Biomathematics, Faculty of Pharmacy and Biology, University of Lille, Lille, France.

Introduction: Neonatal management of aortic coarctation with ventricular septal defect is still under debate between the one-stage full repair by sternotomy versus the staged repair of the coarctation first by thoracotomy (with or without banding the pulmonary artery) followed later by subsequent closure of the ventricular septal defect.

Evidence Acquisition: The aim of this review was to synthesize the evidence in literature since 1980 for the neonatal population. A meta-analysis compared mortality between the two strategies. Read More

Int J Cardiovasc Imaging 2020 Apr 28. Epub 2020 Apr 28.

Post Graduate Institute of Medical Education & Research, Chandigarh, India.

MedEdPORTAL 2020 Mar 20;16:10889. Epub 2020 Mar 20.

Associate Professor of Clinical Pediatrics, Department of Pediatrics, Division of Pediatric Critical Care Medicine, New York Presbyterian-Weill Cornell Medical Center.

Introduction: Critical cardiopulmonary events arising from congenital or acquired heart diseases are infrequent in some pediatric critical care units but can be associated with significant morbidity and mortality when encountered. We developed four simulation cases for interprofessional pediatric critical care teams (fellows, residents, and nurses) to provide participants with high-acuity cardiopulmonary scenarios in safe learning environments. The included cases were coarctation of the aorta, Kawasaki disease, myocarditis, and tetralogy of Fallot. Read More

Catheter Cardiovasc Interv 2020 Apr 27. Epub 2020 Apr 27.

Division of Cardiology, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA.

Objectives: To evaluate short- and middle-term outcomes after transcatheter patent ductus arteriosus (TC-PDA) closure in small infants, specifically device-related left pulmonary artery (LPA) stenosis and aortic coarctation, risk factors, and changes over time.

Background: Recent studies have demonstrated successful transcatheter PDA (TC-PDA) closure in small infants. LPA stenosis and aortic coarctation have been seen after TC-PDA, but it is not clear whether device-related LPA/aortic obstruction persists. Read More

Vasc Endovascular Surg 2020 Jul 26;54(5):463-466. Epub 2020 Apr 26.

Department of Cardiology, Mater Misericordiae University Hospital, Dublin, Ireland.

Aortic isthmic atresia is a severe form of aortic coarctation where there is loss of luminal communication at the aortic isthmus. The primary approach for correcting aortic isthmic atresia has been surgical repair of the coarctation. A small number of case series have shown that percutaneous correction of aortic isthmic atresia is possible. Read More

J Cardiovasc Med (Hagerstown) 2020 Jul;21(7):517-528

Department of Cardiology and Cardiosurgery, Bambino Gesù Children's Hospital, IRCCS.

Aim: A validated algorithm for automatic aortic arch measurements in aortic coarctation (CoA) patients could standardize procedures for clinical planning.

Methods: The model-based assessment of the aortic arch anatomy consisted of three steps: first, machine-learning-based algorithms were trained on 212 three-dimensional magnetic resonance (MR) data to automatically allocate the aortic arch position in patients and segment the aortic arch; second, for each CoA patient (N = 33), the min/max aortic arch diameters were measured using the proposed software, manually and automatically, from noncontrast-enhanced three-dimensional steady-state free precession MRI sequence at five selected sites and compared ('internal comparison' referring to the same environment); third, moreover, the same min/max aortic arch diameters were compared, obtaining them independently, manually from common MR management software (MR Viewforum) and automatically from the model (external comparison). The measured sites were: aortic sinus, sino-tubular junction, mid-ascending aorta, transverse arch and thoracoabdominal aorta at the level of the diaphragm. Read More

Am J Med Genet A 2020 06 22;182(6):1342-1350. Epub 2020 Apr 22.

Pediatric Cardiology and Cardiac Arrhythmias Unit, Department of Pediatric Cardiology and Cardiac Surgery, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy.

Morbidity and mortality in Down syndrome (DS) are mainly related to congenital heart defects (CHDs). While CHDs with high prevalence in DS (typical CHDs), such as endocardial cushion defects, have been extensively described, little is known about the impact of less common CHDs (atypical CHDs), such as aortic coarctation and univentricular hearts. In our single-center study, we analyzed, in observational, retrospective manner, data regarding cardiac features, surgical management, and outcomes of a cohort of DS patients. Read More