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Heart 2020 Jun 29. Epub 2020 Jun 29.

Anaesthesiology & Critical Care, Jawaharlal Institute of Post-graduate Medical Education and Research (JIPMER), Puducherry, India.

Objective: To assess the incidence of adverse cardiac events in pregnant women with rheumatic valvular heart disease (RHD) and to derive a clinical risk scoring for predicting it.

Methods: This is an observational study involving pregnant women with RHD, attending a tertiary centre in south India. Data regarding obstetric history, medical history, maternal complications and perinatal outcome till discharge were collected. Read More

Acta Cardiol 2020 Jun 29:1-6. Epub 2020 Jun 29.

Department of Cardiology, Yüzüncü Yıl University, Van, Turkey.

Performing percutaneous mitral commissurotomy (PMC) in the women with asymptomatic severe mitral stenosis (MS) who plan a pregnancy is recommended. However the data regarding this recommendation is limited in the literature. We aimed to investigate maternal and fetal outcomes of women with asymptomatic severe MS who underwent PMC before a planned pregnancy. Read More

Echocardiography 2020 Jun 28. Epub 2020 Jun 28.

Echocardiography Laboratory, National Institute of Cardiology of Mexico, "Ignacio Chávez", México City, México.

We present the case of an 18-year-old man with aborted sudden cardiac death. His initial echocardiogram suggested an anomalous origin of the left coronary artery from the pulmonary artery. Diagnosis was confirmed with coronary angiography. Read More

Int J Cardiol 2020 Jun 25. Epub 2020 Jun 25.

Heart Center Leipzig at University Leipzig, Department of Internal Medicine/Cardiology, Germany. Electronic address:

Background Pathophysiological differences in relief of pulmonary stenosis (PS) as opposed to pulmonary regurgitation (PR) by percutaneous pulmonary valve implantation (PPVI) remain elusive, but might impact current assessment of procedural success and ultimately indications. Methods Invasive pressure measurements, cardiac magnetic resonance imaging and cardiopulmonary exercise testing were performed before pre-stenting (BMS), after BMS and after PPVI in patients with either PS or PR. Results In PS (n = 14), BMS reduced the right ventricular (RV) to systemic pressure ratio (0. Read More

Heart Vessels 2020 Jun 26. Epub 2020 Jun 26.

Department of Cardiology and Angiology, University Hospital of Tübingen, Tübingen, Germany.

Permanent pacemaker implantation (PPI) is a widely recognized complication associated with TAVI (incidence up to 20%). Smaller registries have identified several variables associated with PPI. The objective was to validate patient- and transcatheter aortic valve implantation (TAVI)-related procedural variables associated with PPI. Read More

Saudi J Med Med Sci 2020 May-Aug;8(2):112-117. Epub 2020 Apr 17.

The Speech and Hearing Center, School of Communication, Louisiana Tech University, Ruston, Louisiana, USA.

Background: Frequencies of normal and abnormal heart sounds have previously been reported, but the acoustic analyses of the frequency responses of conventional and amplified stethoscopes for different heart sounds have not yet been reported.

Objectives: To compare the acoustic analysis of frequency responses of three stethoscopes (conventional and amplified) for measuring simulated heart sounds.

Materials And Methods: This exploratory study used Starkey SLI-ST3, Cardionics E-Scope II (both electronic) and Littmann Classic S. Read More

Can J Cardiol 2020 Jun 22. Epub 2020 Jun 22.

Division of Pediatric Cardiology, Department of Pediatrics, Sainte-Justine UHC, University of Montreal. Electronic address:

Survival of infants born with congenital heart disease (CHD) is improving tremendously and although most of them have mild lesions, others may be considered as only being palliated and undergo many medical or surgical interventions. Patients with CHD will be exposed to the same problematic of the modern lifestyle such as increased prevalence of obesity, decreased physical activities and exposure to smoking which leads to acquired cardiovascular disease. We specifically looked into specific cardiovascular risk factors such as: malnutrition, smoking exposure, hypertension, integrity of the coronary and systemic arteries, thromboembolism, ventricular dysfunction, inflammation, and arrhythmias. Read More

CASE (Phila) 2020 Jun 12;4(3):152-154. Epub 2020 Apr 12.

Department of Cardiovascular Medicine, West Virginia University Heart and Vascular Institute, Morgantown, West Virginia.

Acta Med Litu 2020 ;27(1):10-16

Children's Clinical University Hospital, Department of Paediatric Cardiology and Cardiac Surgery, Riga, Latvia.

Transcatheter pulmonary valve implantation has been a well-known method for more than a decade, but there are still many challenging cases when a personalized solution is needed. We report a case of a 15-year-old female patient with tetralogy of Fallot, who underwent a  surgical correction during infancy. Because of progressive pulmonary regurgitation, stenosis, and right ventricle dilatation, transcatheter pulmonary valve implantation in the native right ventricle outflow tract (RVOT) using Edwards SAPIEN valve was performed. Read More

Birth Defects Res 2020 Jun;112(10):725-731

Pediatric Cardiology, Department of Pediatrics, Obstetrics and Gynecology, Sapienza University of Rome, Rome, Italy.

Background: RASopathies are a set of relatively common autosomal dominant clinically and genetically heterogeneous disorders. Cardiac outcomes in terms of mortality and morbidity for common heart defects (such as pulmonary valve stenosis and hypertrophic cardiomyopathy) have been reported. Nevertheless, also Atypical Cardiac Defects (ACDs) are described. 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

Clin Interv Aging 2020 27;15:755-762. Epub 2020 May 27.

Department of Orthopedics, Guangdong Provincial People's Hospital, Guangdong Academy of Medical Sciences;School of Medicine, South China University of Technology, Guangzhou, People's Republic of China.

Introduction: Transthoracic echocardiography (TTE) is a common cardiac screening test before hip fracture surgery. However, the general TTE test delays surgery, so it would be meaningful if we could simplify the TTE by only assessing cardiac abnormality specifically. Therefore, we aimed to establish the most clinically relevant abnormality by comparing the predictive value of each major cardiac abnormality in postoperative cardiac complications and mortality in elderly hip fracture patients. Read More

Curr Probl Cardiol 2020 May 19:100616. Epub 2020 May 19.

John Ochsner Heart and Vascular Institute, Ochsner Clinic Foundation, New Orleans, LA; The University of Queensland Ochsner Clinical School, Faculty of Medicine, The University of Queensland, New Orleans, LA.

Background: Pulmonary angioplasty has been performed in patients with sarcoidosis-associated pulmonary hypertension (SAPH) but most evidence comes from case reports and small case series. Overall outcomes remain unclear. We conducted an individual participant data (IPD) meta-analysis of baseline, procedural, and outcome data of pulmonary angioplasty in patients with SAPH. Read More

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

Catheter Cardiovasc Interv 2020 Jun 10. Epub 2020 Jun 10.

Centre for Heart Valve Innovation, St Paul's Hospital, University of British Columbia, Vancouver, Canada.

Background: While slow gait speed is known to be associated with poor outcomes in patients at high surgical risk who undergo transcatheter aortic valve replacement (TAVR), the prognostic significance of slow gait speed in intermediate risk TAVR patients is poorly understood.

Objectives: We assessed the association between baseline 6-min walk test (6MWT) performance and both 2-year mortality and health status in intermediate risk patients undergoing TAVR as a part of the PARTNER II/S3i studies.

Methods: The association of baseline 6MWT with mortality over 2-years after TAVR was examined using Cox regression; both unadjusted and adjusted for age, left ventricular ejection fraction, coronary artery disease, pulmonary disease, renal insufficiency, and STS score. Read More

Am J Transplant 2020 Jun 7. Epub 2020 Jun 7.

The University of Florida, Department of Surgery, Division of Thoracic and Cardiovascular Surgery, Florida, USA.

End-stage lung disease and advanced cardiac conditions are frequently seen together and represent a clinical dilemma. Even though both issues may be amenable to surgical management, combining lung transplantation with surgical valve repair is rarely done and theoretically associated with increased morbidity and mortality risks, especially in elderly patients. Here, we describe 2 patients presenting with end-stage lung disease and significant aortic stenosis who were successfully bridged to lung transplantation via transcatheter aortic valve replacement. Read More

Int J Surg Case Rep 2020 May 30;72:32-36. Epub 2020 May 30.

Sri Jayadeva Institute of Cardiovascular Sciences and Research, Bangalore, Karnataka, India.

Introduction: Noonan syndrome is the second most common syndromic cause of congenital heart disease. Most patients have an autosomal dominant inheritance, but some cases may be sporadic. Pulmonary stenosis is the most common cardiac manifestation in Noonan syndrome, associated with the atrial septal defect and hypertrophic cardiomyopathy. Read More

Int J Cardiol 2020 Apr 13. Epub 2020 Apr 13.

Quebec Heart and Lung Institute, Laval University, Quebec City, Quebec, Canada. Electronic address:

Background: Few and controversial data exist on the outcomes of patients with paradoxical low-flow, low-gradient aortic stenosis (PLFLG-AS) following transcatheter aortic valve replacement (TAVR). This study aims to better characterize clinical outcomes and predictors of treatment futility in PLFLG-AS patients undergoing TAVR.

Methods: In this multicenter study, 318 patients with PLFLG-AS undergoing TAVR were categorized according to treatment futility, defined as all-cause mortality, poor functional status (NYHA class III-IV) or deterioration in functional class at 1-year follow-up. Read More

Int J Cardiovasc Imaging 2020 Jun 4. Epub 2020 Jun 4.

Department of Cardiology, University Hospital Leipzig, Liebigstraße 20, 04103, Leipzig, Germany.

Purpose: In echocardiography the severity of aortic stenosis (AS) is defined by effective orifice area (EOA), mean pressure gradient (mPG) and transvalvular flow velocity (maxV). The hypothesis of the present study was to confirm the pathophysiological presence of combined left ventricular hypertrophy (LVH), diastolic dysfunction (DD) and pulmonary artery hypertension (PAH) in patients with "pure" severe AS.

Methods And Results: Patients (n = 306) with asymptomatic (n = 133) and symptomatic (n = 173) "pure" severe AS (mean age 78 ± 9. Read More

Pediatr Radiol 2020 Jul 4;50(8):1030-1040. Epub 2020 Jun 4.

Department of Clinical Radiology, Sidra Medicine, Weill Cornell Medicine, Doha, Qatar.

There is a broad spectrum of congenital anomalies of the central pulmonary arteries including abnormalities of development, origin, course and caliber. These anomalies incorporate simple lesions such as isolated pulmonary valve stenosis to very complex anomalies with many associated abnormalities. Part 1 and Part 2 of this review describe the range of anatomical variations that are encountered as well as important aspects of anatomy, physiology and surgical correction. Read More

J Cardiothorac Surg 2020 Jun 4;15(1):124. Epub 2020 Jun 4.

Department of Cardiology, Qingyuan People's Hospital, the Sixth Affiliated Hospital of Guangzhou Medical University, 22 Shuguang Second Road, Qingcheng, Qingyuan, Guangdong, 511500, People's Republic of China.

Background: Woven coronary artery (WCA) is an extremely rare congenital anomaly in which a part of epicardial coronary artery is divided into thin channels, that twist along the axis of the coronary arteries and then merge again as the main coronary lumen. This anomaly is regarded as a benign condition because the blood flow is normal. Very few cases of WCA have been reported. Read More

Eur J Cardiovasc Nurs 2020 Jun 4:1474515120934057. Epub 2020 Jun 4.

Centre for Heart Valve Innovation, St. Paul's Hospital, University of British Columbia, Vancouver Canada.

The COVID-19 pandemic continues to significantly impact the treatment of people living with aortic stenosis, and access to transcatheter aortic valve implantation. Transcatheter aortic valve implantation (TAVI) programmes require unique coordinated processes that are currently experiencing multiple disruptions and are guided by rapidly evolving protocols. We present a series of recommendations for TAVI programmes to adapt to the new demands, based on recent evidence and the international expertise of nurse leaders and collaborators in this field. Read More

Curr Opin Lipidol 2020 Jun 1. Epub 2020 Jun 1.

Princip D'Espanya, Miami Platja, Tarragona, Spain.

Purpose Of Review: Studies have shown the three-member paraoxonase (PON) multigene family to be involved in the development of a large variety of diseases with an inflammatory component. Environmental factors such as lifestyle-related factors differ widely between populations and it is important to consider that their impacts may be exerted through the epigenetic mechanisms, which connect genes, the environment and disease development and are a potential therapeutic avenue.

Recent Findings: In the review period, very little was published on epigenetics of PON2 or PON3, mostly on their diagnostic value in cancer by measuring methylation levels of these genes. Read More

Pediatr Radiol 2020 Jul 30;50(8):1022-1029. Epub 2020 May 30.

Department of Clinical Radiology, Sidra Medicine, Weill Cornell Medicine, Doha, Qatar.

There is a broad spectrum of congenital anomalies of the central pulmonary arteries including abnormalities of development, origin, course and caliber. These anomalies incorporate simple lesions such as isolated pulmonary valve stenosis to very complex anomalies with many associated abnormalities. Part 1 and Part 2 of this review describe the range of anatomical variations that are encountered as well as important aspects of anatomy, physiology and surgical correction. Read More

Cureus 2020 Apr 25;12(4):e7829. Epub 2020 Apr 25.

Internal Medicine, Citrus Memorial Hospital, Inverness, USA.

Rhodococcus equi (R. equi) is a rare zoonotic organism that is found in the feces of grazing animals and in farm soil. It typically causes pulmonary disease, but it can also cause extrapulmonary disease. Read More

Multimed Man Cardiothorac Surg 2020 May 26;2020. Epub 2020 May 26.

Department of Cardiothoracic Surgery Narayana Institute of Cardiac Sciences Bengaluru, India.

Right ventricle to pulmonary artery conduits such as homografts and pre-manufactured synthetic conduits are widely employed in the present era of complex congenital cardiac surgeries for disorders involving right ventricle - pulmonary artery discontinuity and major coronary artery crossing the right ventricular outflow tract. The key drawback of homograft conduits is the need for reoperation to replace them as a result of degeneration over time or in cases where a child has outgrown the conduit and cost is a major drawback to using commercially available conduits. The advantages of expanded polytetrafluoroethylene are its long-term durability in terms of conduit calcification/degeneration and valve stenosis/regurgitation and its cost-effectiveness. Read More

Int J Cardiovasc Imaging 2020 May 26. Epub 2020 May 26.

Heart and Vascular Institute, Einstein Medical Center, Philadelphia, PA, USA.

Mitral annular calcification (MAC) is increasingly encountered, particularly among the elderly and those with chronic kidney disease, and is often associated with a transvalvular gradient. In contrast to rheumatic mitral stenosis relatively little is known about mitral stenosis due to MAC. We aimed to clarify whether exercise limitation in this group is primarily due to valvular obstruction or ventricular dysfunction resulting from multiple comorbidities. Read More

J Cardiol 2020 May 22. Epub 2020 May 22.

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

Background: Percutaneous balloon pulmonary valvuloplasty (PBPV) is the procedure of choice for uncomplicated severe or symptomatic pulmonary stenosis. Echocardiography (echo)-guided PBPV can completely avoid the use of radiation and contrast agents compared to fluoroscopy-guided PBPV. Although we have confirmed that echo-guided PBPV is feasible in humans, the poor visibility of the traditional catheter under echo greatly limits the promotion of this new technology. Read More

J Card Surg 2020 May 22. Epub 2020 May 22.

Department of Cardiovascular Surgery, Keio University School of Medicine, Tokyo, Japan.

Marked thrombocytopenia causes significant bleeding in cardiovascular surgery. Herein, we describe the case of a 47-year-old woman with immune thrombocytopenia who underwent successful pulmonary valve replacement for pulmonary valve regurgitation and stenosis after complete repair of tetralogy of Fallot. Her platelet count decreased significantly to less than 5 × 10 /L on postoperative day 3, thus multiple platelet transfusions were given. Read More

J Atr Fibrillation 2019 Dec 31;12(4):2143. Epub 2019 Dec 31.

Lecturers, Department of Pharmacology, Nobel Medical College Teaching Hospital, Biratnagar, Nepal.

Introduction: Rheumatic heart disease (RHD) is one of the common causes of atrial fibrillation (AF) is associated with significant morbidity and mortality. There is a lack of data on the prevalence of AF and factors associated with increased risk of AF in patients with RHD from Nepal.

Methods: A total of 120 patients who received care at Nobel Medical College Teaching Hospital from January 2018 to February 2019 with a diagnosis of RHD with AF were enrolled. 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

G Ital Cardiol (Rome) 2020 Jun;21(6):469-477

Centro Nazionale per la Salute Globale, Istituto Superiore di Sanità, Roma.

Background: The OBSERVANT study evaluated the medium and long-term outcomes in patients undergoing transcatheter aortic valve implantation (TAVI) versus surgical aortic valve replacement obtaining comparative effectiveness results at least for the first-generation devices. The OBSERVANT II study enrolled a new series of TAVI procedures to evaluate whether the use of new-generation devices has modified outcome differences recorded in the OBSERVANT study. The aim of this paper is to describe the clinical characteristics of the OBSERVANT II population and compare them with those of the OBSERVANT population. Read More

Can J Cardiol 2020 Feb 8. Epub 2020 Feb 8.

Cardiology Division, Kantonsspital, St Gallen, Switzerland.

Background: An echocardiographic 5-stage classification of cardiac damage in aortic stenosis (AS) has been shown to provide prognostic information. We aimed to create an analogous classification based on invasive hemodynamics.

Methods: We studied 421 patients (age 75 ± 10 years, 59% men) with severe AS with complete invasive hemodynamics obtained before aortic valve replacement (AVR). Read More

Ann Thorac Surg 2020 May 11. Epub 2020 May 11.

Department of Cardiovascular Surgery, West China School of Medicine/West China Hospital of Sichuan University. Electronic address:

Background: Evidence about safety and efficacy of transcatheter aortic valve replacement (TAVR) remains limited for patients with aortic regurgitation (AR).

Methods: From the Safety & Efficacy of the J-Valve Ausper System in Patients with Severe Aortic Stenosis and/or Aortic Regurgitation Registry, the clinical outcomes of patients with AR from West China Hospital were analyzed.

Results: A total of 134 patients with a mean Society of Thoracic Surgeons risk score of 9. Read More

Surg Case Rep 2020 May 13;6(1):101. Epub 2020 May 13.

Division of Thoracic and Cardiovascular Surgery, Niigata University Graduate School of Medical and Dental Sciences, 1-757, Asahimachi-dori, Niigata City, 951-8510, Japan.

Background: In a Fontan candidate, univentricular pacing may cause delay in interventricular conduction, which induces asynchronous contraction. Cardiac resynchronization therapy is expected to be an effective mode of therapy in such a case.

Case Presentation: A 7-month-old girl, diagnosed with dextrocardia, congenitally corrected transposition of the great artery [situs solitus, L-loop, and L-transposition], ventricular septal defect, infundibular and pulmonary valvular stenosis, and straddling of the tricuspid valve, was considered as a candidate for the Fontan procedure. Read More

Pediatr Cardiol 2020 May 11. Epub 2020 May 11.

Department of Paediatrics and Adolescent Medicine, Queen Mary Hospital, The University of Hong Kong, Hong Kong, China.

We determined the prevalence and factors associated with tricuspid regurgitation (TR) in adults with repair of right ventricular (RV) outflow obstruction. A total of 256 patients (128 males) were studied at 25.7 ± 7. Read More

Kyobu Geka 2020 Feb;73(2):99-103

Department of Cardiovascular and Thoracic Surgery, Ehime University, Toon, Japan.

A 47-year-old woman with a history of mitral valve replacement (MVR) through a median sternotomy was admitted to our hospital due to dyspnea on exertion. Echocardiography showed bioprosthetic valve dysfunction with mitral stenosis. Right heart catheter examination revealed severe pulmonary hypertension and right ventricular dysfunction. Read More

Arch Iran Med 2020 May 1;23(5):356-358. Epub 2020 May 1.

Atherosclerosis Research Center, Ahvaz JundiShapur University of Medical Sciences, Ahvaz, Iran.

Double trisomy 48, XXY, +21 or Down-Klinefelter syndrome is a rare occurrence and presents clinical manifestation of trisomy 21 in early life and of Klinefelter syndrome after 10 months of age. The phenotypic and karyotyping characteristics of a 2-month-old boy were reported. He had mild clinical feature of Down syndrome and echocardiographic features of atrioventricular (AV) septal defects with severe pulmonary valve stenosis. Read More

Surg Technol Int 2020 May;36:217-223

Department of Cardiovascular Surgery, Mount Sinai Medical Center, Icahn School of Medicine at Mount Sinai, New York, New York.

Severe tricuspid regurgitation (TR) is associated with significant mortality and morbidities. Currently, surgical tricuspid repair with annuloplasty is the gold standard treatment. However, the prohibitive risks of open surgery and increasing evidence that severe TR should be intervened on early has led to an outburst in the development and evolution of transcatheter tricuspid valve interventions (TTVI). Read More

Int J Cardiol Heart Vasc 2020 Jun 24;28:100517. Epub 2020 Apr 24.

Department of Cardiovascular Surgery, The Sakakibara Heart Institute of Okayama, Japan.

Objective: The aim of this study is to evaluate mitral valve hemodynamics after mitral valve repair for degenerative disease, and seek the impact of type/size of annuloplasty prosthesis on resting cardiac hemodynamics.

Methods: Between October 2012 and June 2019, 301 patients underwent isolated mitral valve repair for degenerative disease were enrolled. Correlation between postoperative mitral hemodynamics and type/size of annuloplasty prosthesis was evaluated. Read More

Cardiol Young 2020 Jun 4;30(6):774-778. Epub 2020 May 4.

Department of Thoracic and Cardiovascular Surgery, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.

Background: A certain degree of pulmonary stenosis after total correction of tetralogy of Fallot has been considered acceptable. But the long-term outcomes are not well understood. We observed the natural course of immediate pulmonary stenosis and investigated related factors for progression. Read More

Am Heart J 2020 Jun 8;224:105-112. Epub 2020 Feb 8.

Department of Cardiology, Duke University Medical Center, Durham, NC. Electronic address:

Background: Patients with bicuspid aortic valve stenosis (BAV) were excluded from all the trileaflet aortic valve stenosis (TAV) pivotal trials, and therefore, their outcomes are not clearly defined. The aim of the study was to evaluate the outcomes of transcatheter aortic valve replacement (TAVR) in patients with BAV and compared them with those of TAV.

Methods: We evaluated the outcomes following TAVR of patients with BAV at our institution between April 2011 and November 2016 and compared them with the outcomes of patients with TAV treated with TAVR. Read More

Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2020 ;23:24-28

Division of Cardiac, Thoracic, and Vascular Surgery, NewYork-Presbyterian/Columbia University Medical Center, Morgan Stanley Children's Hospital of New York, Columbia University College of Physicians and Surgeons, New York, New York. Electronic address:

Valve-sparing techniques for the treatment of Tetralogy of Fallot with pulmonary stenosis have evolved over the past few decades. This article will discuss the rationale for a more aggressive approach to sparing the pulmonary valve, the various techniques available (including commissurotomy, leaflet thinning and debridement, balloon dilation, and finally modified monocusp repair), as well as a systematic approach for employing these techniques based on individual patient anatomy in a manner that takes full advantages of the benefits of the valve-sparing approach while mitigating the risks involved. Read More

Innovations (Phila) 2020 Mar/Apr;15(2):111-113

Duke Congenital Heart Surgery Research & Training Laboratory, Durham, NC, USA.

A 12-year-old, 32 kg male with history of Tetralogy of Fallot status post repair at 8 days of life presented with progressive pulmonary insufficiency and left pulmonary artery stenosis. Surgical options were discussed, and the patient and his family elected to pursue minimally invasive pulmonary valve replacement with left pulmonary artery augmentation through a 5-cm left anterior mini-incision. The procedure was performed without complication, and he was discharged on postoperative day 3. Read More

Intern Med 2020 Apr 30. Epub 2020 Apr 30.

Department of Cardiovascular Medicine, Nagasaki University Graduate School of Biomedical Sciences, Japan.

A 73-year-old Japanese man was admitted with an asymptomatic pulmonary artery aneurysm. However, chest X-ray and contrast-enhanced thoracic computed tomography revealed a protrusion at the second left branch that in fact was a pulmonary artery aneurysm with a diameter of 50 mm. Transesophageal echocardiography showed a bicuspid pulmonary valve, and cardiac catheterization revealed pulmonary stenosis with a pressure gradient of 45 mmHg, but no other heart diseases were noted. Read More

Clin Genet 2020 Jul 19;98(1):56-63. Epub 2020 May 19.

CENTOGENE AG, Rostock, Germany.

Recently, ADAMTS19 was identified as a novel causative gene for autosomal recessive heart valve disease (HVD), affecting mainly the aortic and pulmonary valves. Exome sequencing and data repository (CentoMD) analyses were performed to identify patients with ADAMTS19 variants (two families). A third family was recognized based on cardiac phenotypic similarities and SNP array homozygosity. Read More

J Card Surg 2020 May 17;35(5):1152-1155. Epub 2020 Apr 17.

Department of Anaesthesia and Critical Care, Post Graduate Institute of Medical Education and Research, Chandigarh, India.

Congenital aneurysmal dilatation of coronary artery with coronary cameral fistula is rare in childhood. We report an even rarer association of congenital right coronary artery aneurysm and right coronary artery to right ventricle fistula with bicuspid pulmonary valve stenosis and an intact ventricular septum in a 3-year-old child. Read More