Paul M. Weinberg

Report from The International Society for Nomenclature of Paediatric and Congenital Heart Disease: cardiovascular catheterisation for congenital and paediatric cardiac disease (Part 2 – Nomenclature of complications associated with interventional cardiology)

Interventional cardiology for paediatric and congenital cardiac disease is a relatively young and rapidly evolving field. As the profession begins to establish multi-institutional databases, a universal system of nomenclature is necessary for the field of interventional cardiology for paediatric and congenital cardiac disease. The purpose of this paper is to present the results of the efforts of The International Society for Nomenclature of Paediatric and Congenital Heart Disease to establish a system of nomenclature for cardiovascular catheterisation for congenital and paediatric cardiac disease, focusing both on procedural nomenclature and the nomenclature of complications associated with interventional cardiology. This system of nomenclature for cardiovascular catheterisation for congenital and paediatric cardiac disease is a component of The International Paediatric and Congenital Cardiac Code. This manuscript is the second part of the two-part series. Part 1 covered the procedural nomenclature associated with interventional cardiology as treatment for paediatric and congenital cardiac disease. Part 2 will cover the nomenclature of complications associated with interventional cardiology as treatment for paediatric and congenital cardiac disease.

Report from The International Society for Nomenclature of Paediatric and Congenital Heart Disease: cardiovascular catheterisation for congenital and paediatric cardiac disease (Part 1 - Procedural nomenclature)

Interventional cardiology for paediatric and congenital cardiac disease is a relatively young and rapidly evolving field. As the profession begins to establish multi-institutional databases, a universal system of nomenclature is necessary for the field of interventional cardiology for paediatric and congenital cardiac disease. The purpose of this paper is to present the results of the efforts of The International Society for Nomenclature of Paediatric and Congenital Heart Disease to establish a system of nomenclature for cardiovascular catheterisation for congenital and paediatric cardiac disease, focusing both on procedural nomenclature and on the nomenclature of complications associated with interventional cardiology. This system of nomenclature for cardiovascular catheterisation for congenital and paediatric cardiac disease is a component of The International Paediatric and Congenital Cardiac Code. This manuscript is the first part of a two-part series. Part 1 will cover the procedural nomenclature associated with interventional cardiology as treatment for paediatric and congenital cardiac disease. This procedural nomenclature of The International Paediatric and Congenital Cardiac Code will be used in the IMPACT Registry™ (IMproving Pediatric and Adult Congenital Treatment) of the National Cardiovascular Data Registry® of The American College of Cardiology. Part 2 will cover the nomenclature of complications associated with interventional cardiology as treatment for paediatric and congenital cardiac disease.

Mitral valve dysplasia syndrome: A unique form of left-sided heart disease

BACKGROUNDnMitral valve dysplasia syndrome is a unique form of left-sided heart disease characterized by aortic outflow hypoplasia, dilated left ventricle, dysplastic/incompetent mitral valve, and a restrictive/intact atrial septum. Patients with this constellation of abnormalities have been managed in a variety of ways with overall poor outcomes.nnnMETHODSnWe performed a retrospective review of all patients with mitral valve dysplasia syndrome to identify fetal echocardiographic markers predictive of outcomes.nnnRESULTSnMitral valve dysplasia syndrome was identified in 10 fetuses. Fetal left heart dilation and abnormal pulmonary venous flow were associated with increased mortality. Seven fetuses had abnormal pulmonary venous Doppler patterns; 3 had a unique double-reversal flow pattern. Severe fetal left heart dilation (left heart/right heart area ratio > 1.5) was present in 5. Prenatal intervention was performed on 3 fetuses: balloon aortic valvuloplasty (nxa0=xa02) and balloon atrial septostomy (nxa0=xa01). Of the 3, one died in utero and neither survivor underwent a 2-ventricle repair. Five patients required an immediate postnatal intervention to open the atrial septum. The overall mortality was 50%.nnnCONCLUSIONSnMitral valve dysplasia syndrome is a unique form of congenital heart disease with severe aortic stenosis but normal or enlarged left ventricle secondary to primary mitral valve disease. Increased left heart size and pulmonary vein Doppler patterns are predictive of postnatal outcome. Despite the presence of a dilated left ventricle, postnatal management with staged single ventricle palliation may be the most effective strategy.

Cardiac MRI and CT: Differentiation of Normal Ostium and Intraseptal Course From Slitlike Ostium and Interarterial Course in Anomalous Left Coronary Artery in Children

OBJECTIVEnAnomalous left coronary artery from the inappropriate aortic sinus with intraseptal course is generally benign but can be confused on imaging studies with the potentially lethal interarterial, intramural anomalous left coronary artery. The purpose of this study was to assess normal ostial morphologic features and intraseptal course using cardiac MRI and CT in pediatric patients with intraseptal anomalous left coronary artery.nnnMATERIALS AND METHODSnA retrospective review was conducted of the medical records of 14 children with the diagnosis of intraseptal anomalous left coronary artery between November 2009 and March 2013. Coronary artery origin and course were evaluated with cardiac MRI or CT, and 3D assessment of coronary ostial morphologic features was performed with virtual angioscopy.nnnRESULTSnThe patient ages ranged from 5 to 18 years at diagnosis; 10 (71.4%) were boys. The right and left coronary origins were the right sinus of Valsalva as a common origin (n = 9) or a single coronary artery (n = 5). Anomalous intraseptal left main coronary was found in 13 patients, and one patient had anomalous left anterior descending with retroaortic circumflex coronary artery. Anomalous coronary ostia were round and without stenosis in all studies. The anomalous vessel was identified with echocardiography, but the anomalous left coronary artery was not delineated, and a normal ostium was not adequately portrayed in any instance.nnnCONCLUSIONnBy use of cardiac MRI and CT, the anomalous course of round coronary ostia was confirmed and visualized in a pediatric cohort with intraseptal anomalous left coronary artery. The data provide the basis for understanding the benign clinical course and showing that surgery is unnecessary for this coronary anomaly.

Differential Branch Pulmonary Artery Regurgitant Fraction Is a Function of Differential Pulmonary Arterial Anatomy and Pulmonary Vascular Resistance

OBJECTIVESnWe sought to investigate whether differential branch pulmonary artery (BPA) regurgitation correlates with differences in BPA anatomy and physiology.nnnBACKGROUNDnPatients with repaired conotruncal anomalies such as Tetralogy of Fallot frequently have residual BPA stenosis or BPA size differences. Previous reports have demonstrated an increased left pulmonary artery (LPA) regurgitant fraction (RF) in these patients.nnnMETHODSnWe retrospectively reviewed 76 consecutive cardiac magnetic resonance (CMR) studies for BPA size and phase-contrast magnetic resonance data, including 13 consecutive patients who underwent both CMR and catheterization.nnnRESULTSnThirty of the 76 patients had either BPA stenosis or significant size discrepancy. Whereas previous studies had shown an increased RF in the LPA, patients with BPA stenosis or size discrepancy showed no significant difference between right and left BPA RF (30% vs. 30%, p = 0.985). However, there was a significantly increased RF of the larger versus smaller BPA (39% vs. 21%, p < 0.001), resulting in an insignificant deviation from normal fractional flow distribution (RPA 63% vs. LPA 37%; normal net fractional flow distribution RPA 55% vs. LPA 45%). Retrospective review of patients who underwent both CMR and catheterization provides support for the preceding findings and validates differential BPA RF as strongly correlating with differential pulmonary vascular resistance (PVR) (r = 0.8364, p < 0.001).nnnCONCLUSIONSnBPA RF is a function of the relative PVR and the presence of BPA stenosis or size discrepancy. Contrary to prior reports, the LPA RF is only elevated in patients with relatively equal sized BPAs. In the setting of BPA stenosis or size discrepancy the larger BPA has a relatively increased RF and PVR. Therefore, the differential RF is an important tool for screening patients with unilateral stenosis for contralateral increases in PVR that cannot be identified with net flows alone. This can affect the indication and timing for BPA intervention.

Conjoined Hearts in Thoracopagus Twins

This study aimed to identify the anatomic and pathologic structural cardiac abnormalities in conjoined twins and to focus on those that have prevented the successful separation of conjoined hearts. A retrospective review was undertaken to examine consecutive cases of thoracopagus conjoined twins with conjoined hearts evaluated at The Children’s Hospital of Philadelphia from 1 January 1980 through 6 October 2008. The records included autopsy and surgical findings as well as clinical reports. The study group included nine sets of conjoined twins with a mean gestational age at birth of 33.8xa0±xa05.5xa0weeks. Three twin pairs were stillborn. Five twin pairs died afterward. One pair died of cardiopulmonary failure. The median age at death was 22xa0days (range, 0–345xa0days). Major congenital heart disease was present in 94.4% (17/18) of the hearts, and 72.2% (13/18) of the hearts had single-ventricle physiology. Total anomalous pulmonary venous return occurred in 39% (7/18) of the cases. The clinical outcome for thoracopagus twins with conjoined hearts remains poor because of inability to separate conjoined and single ventricles. Surgical nonintervention and palliative care should be strongly considered for these patients.

Task Force 2: Pediatric Cardiology Fellowship Training in Noninvasive Cardiac Imaging

### 1.1 Document Development ProcessnnThe Society of Pediatric Cardiology Training Program Directors (SPCTPD) board assembled a steering committee that nominated 2 chairs, 1 SPCTPD steering committee member, and 4 additional experts from a wide range of program sizes, geographic regions, and

Identifying Abnormal Ostial Morphology in Anomalous Aortic Origin of a Coronary Artery

BACKGROUNDnAnomalous origin of a coronary artery from the contralateral sinus of Valsalva is associated with exercise-induced ischemia and sudden death. That is thought to be due to aortic enlargement in patients with an elliptical ostium. We hypothesize that virtual angioscopy can identify abnormal coronary ostial morphology in these patients.nnnMETHODSnWe retrospectively analyzed 55 consecutive pediatric coronary artery magnetic resonance imaging studies from January 2006 to January 2010 with the diagnosis of anomalous right (n = 20), or left (n = 7) coronary artery, or normal coronary origins (n = 28). One postmortem heart specimen with anomalous left coronary artery was imaged and analyzed to validate our technique. Virtual angioscopy analysis was used for visualization and measurement of the coronary ostia.nnnRESULTSnDistinct aortic origins of the right and left coronaries were seen in all 55 studies. An elliptical orifice with a longer superior-inferior dimension was seen in all anomalous ostia, in contrast to a circular ostium in all normal origins. That was quantified in anomalous ostia with a long-axis to short-axis ratio of 2.5 ± 0.5 (right) and 2.4 ± 0.5 (left) compared with 1.1 ± 0.2 (right) and 1.0 ± 0.3 (left) in controls (p < 0.001 for right and left ostia comparisons). Ostial morphology was confirmed in all 9 patients who underwent operative repair and in 1 patient at autopsy.nnnCONCLUSIONSnVirtual angioscopy identifies abnormal ostial morphology in anomalous coronary artery patients, which is important for characterizing the diagnosis of patients who may be at risk for sudden death.

Hypoplastic Left Ventricle and Scimitar Syndrome

Hypoplastic left ventricle with scimitar syndrome is a rare combination of anatomic lesions. Five patients with this anatomy have been followed up at our institution. Four of these patients are alive: 2 after heart transplant, 1xa0underwent a Norwood operation, and 1xa0has survived staged palliation, culminating in a Fontan operation.

Absent Pulmonary Valve, Tricuspid Atresia, and Congenital Heart Block

We describe management of a patient with a prenatal diagnosis of absent pulmonary valve, tricuspid atresia, ventricular septal defect, and congenital heart block. Initial treatment consisted of temporary pacemaker implantation, and subsequent palliation included a central shunt during the neonatal period and placement of a permanent pacemaker. At seven months of age, a bidirectional Glenn anastomosis was performed. Cardiac catheterization revealed high cavopulmonary pressures and ventricular dysfunction precluding Fontan completion. Heart transplantation was performed at 3.75 years of age. The patient is alive and well 26 months posttransplantation.