Congenital absence of coronary ostia in a single/common coronary system.

Coronary ostial atresia seen with pulmonary atresia and coronary-cameral fistulae or, more rarely, in isolation manifested as left main coronary artery atresia, is well described. We describe the clinical course and post-mortem findings in a neonate who suffered a fatal cardiac arrest and was found to have congenital absence of both coronary ostia in a single/common coronary system.

Biventricular Repair in Interrupted Aortic Arch Type C With Aortic Atresia.

We report a case of interrupted aortic arch type C with aortic atresia and a ventricular septal defect with two well-developed ventricles, who underwent a successful single-stage biventricular repair with the modified Yasui procedure and arch reconstruction. Angiography done during conduit revision showed bilateral brachiocephalic trunks with high branching. The child is doing well six years after the initial operation.

Classification of Ventricular Septal Defects for the Eleventh Iteration of the International Classification of Diseases-Striving for Consensus: A Report From the International Society for Nomenclature of Paediatric and Congenital Heart Disease.

The definition and classification of ventricular septal defects have been fraught with controversy. The International Society for Nomenclature of Paediatric and Congenital Heart Disease is a group of international specialists in pediatric cardiology, cardiac surgery, cardiac morphology, and cardiac pathology that has met annually for the past 9 years in an effort to unify by consensus the divergent approaches to describe ventricular septal defects. These efforts have culminated in acceptance of the classification system by the World Health Organization into the 11th Iteration of the International Classification of Diseases. The scheme to categorize a ventricular septal defect uses both its location and the structures along its borders, thereby bridging the two most popular and disparate classification approaches and providing a common language for describing each phenotype. Although the first-order terms are based on the geographic categories of central perimembranous, inlet, trabecular muscular, and outlet defects, inlet and outlet defects are further characterized by descriptors that incorporate the borders of the defect, namely the perimembranous, muscular, and juxta-arterial types. The Society recognizes that it is equally valid to classify these defects by geography or borders, so the emphasis in this system is on the second-order terms that incorporate both geography and borders to describe each phenotype. The unified terminology should help the medical community describe with better precision all types of ventricular septal defects.

Nomenclature for congenital and paediatric cardiac disease: the International Paediatric and Congenital Cardiac Code (IPCCC) and the Eleventh Iteration of the International Classification of Diseases (ICD-11).

An internationally approved and globally used classification scheme for the diagnosis of CHD has long been sought. The International Paediatric and Congenital Cardiac Code (IPCCC), which was produced and has been maintained by the International Society for Nomenclature of Paediatric and Congenital Heart Disease (the International Nomenclature Society), is used widely, but has spawned many "short list" versions that differ in content depending on the user. Thus, efforts to have a uniform identification of patients with CHD using a single up-to-date and coordinated nomenclature system continue to be thwarted, even if a common nomenclature has been used as a basis for composing various "short lists". In an attempt to solve this problem, the International Nomenclature Society has linked its efforts with those of the World Health Organization to obtain a globally accepted nomenclature tree for CHD within the 11th iteration of the International Classification of Diseases (ICD-11). The International Nomenclature Society has submitted a hierarchical nomenclature tree for CHD to the World Health Organization that is expected to serve increasingly as the "short list" for all communities interested in coding for congenital cardiology. This article reviews the history of the International Classification of Diseases and of the IPCCC, and outlines the process used in developing the ICD-11 congenital cardiac disease diagnostic list and the definitions for each term on the list. An overview of the content of the congenital heart anomaly section of the Foundation Component of ICD-11, published herein in its entirety, is also included. Future plans for the International Nomenclature Society include linking again with the World Health Organization to tackle procedural nomenclature as it relates to cardiac malformations. By doing so, the Society will continue its role in standardising nomenclature for CHD across the globe, thereby promoting research and better outcomes for fetuses, children, and adults with congenital heart anomalies.

Pulmonary atresia with intact ventricular septum and coronary cameral fistulae: an unusual finding of a subsystemic right ventricle.

We report the case of a neonate with pulmonary atresia with intact ventricular septum and coronary cameral fistulae despite having a subsystemic right ventricle. We review the literature on coronary cameral fistulae in this disease and right ventricle-dependent coronary circulation. We discuss the potential consequences of this physiology, including risk of adverse cardiovascular events that may impact risk stratification and surgical palliation.

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

BACKGROUND: Anomalous 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. METHODS: We 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. RESULTS: Distinct 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. CONCLUSIONS: Virtual 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.

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.

Cardiac MRI and CT: differentiation of normal ostium and intraseptal course from slitlike ostium and interarterial course in anomalous left coronary artery in children.

OBJECTIVE: Anomalous 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. MATERIALS AND METHODS: A 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. RESULTS: The 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. CONCLUSION: By 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.

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, 1 underwent a Norwood operation, and 1 has survived staged palliation, culminating in a Fontan operation.

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 33.8 ± 5.5 weeks. Three twin pairs were stillborn. Five twin pairs died afterward. One pair died of cardiopulmonary failure. The median age at death was 22 days (range, 0-345 days). 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.

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

BACKGROUND: Mitral 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. METHODS: We performed a retrospective review of all patients with mitral valve dysplasia syndrome to identify fetal echocardiographic markers predictive of outcomes. RESULTS: Mitral 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 (n = 2) and balloon atrial septostomy (n = 1). 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%. CONCLUSIONS: Mitral 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.

Differential branch pulmonary artery regurgitant fraction is a function of differential pulmonary arterial anatomy and pulmonary vascular resistance.

OBJECTIVES: We sought to investigate whether differential branch pulmonary artery (BPA) regurgitation correlates with differences in BPA anatomy and physiology. BACKGROUND: Patients 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. METHODS: We 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. RESULTS: Thirty 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). CONCLUSIONS: BPA 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.

A novel case of L-transposition with a right-dominant double aortic arch.

We describe a case of congenitally corrected transposition with a double aortic arch. This unique combination of lesions highlights the importance of a complete anatomic assessment prior to referral for surgery.

Pre-Fontan cardiac magnetic resonance predicts post-Fontan length of stay and avoids ionizing radiation.

OBJECTIVE: Patients frequently undergo cardiac catheterization before the Fontan operation because of the limited echocardiographic windows in the region of the superior cavopulmonary connection and branch pulmonary arteries. Patients with obstruction to pulmonary blood flow are at increased risk for prolonged length of hospital stay after the Fontan operation. Cardiac magnetic resonance has unlimited imaging windows and can quantify both the branch pulmonary artery size and net flow distribution and thereby serve as a method for identifying patients at increased risk for prolonged length of stay. METHODS: We retrospectively reviewed 24 cardiac magnetic resonance studies of patients (mean age, 3.1 +/- 1.0 years) referred before the Fontan operation. Cardiac magnetic resonance measured the cross-sectional area and flow to each branch pulmonary artery. Post-Fontan hospital course data were acquired from the medical record. RESULTS: Prolonged length of stay after the Fontan operation is observed among patients with one branch that is less than 25% of the total cross-sectional area (18.0 +/- 5.5 vs 8.2 +/- 3.8 days, P = .01) or with less than 40% flow to one branch (12.5 +/- 6.9 vs 7.6 +/- 1.5 days, P = .04). There is moderate correlation between the total branch pulmonary area and length of stay (r = -0.75). CONCLUSIONS: Cardiac magnetic resonance noninvasively assesses the branch pulmonary area size and flow before the Fontan operation. These data predict which patients are more likely to experience a prolonged hospital course.