Ostium Secundum-type Atrial Septal Defect Research Articles

2q31 microdeletion syndrome with the velocardiofacial phenotype and review of the literature: a case report

BackgroundThe 2q31 deletion results in a distinct phenotype characterized by varying degrees of developmental delay, short stature, facial dysmorphism, and variable limb defects. Dysmorphic features include microcephaly, downslanting palpebral fissures, a long and flat philtrum, micrognathia, and dysplastic, low-set ears. To date, comparative genomic hybridization has identified this deletion in 38 patients. Consequently, additional patients with comprehensive clinical data are required to fully understand the spectrum of clinical manifestation associated with a deletion in the 2q31 cytoband.Case presentationWe present the case of an 8-year-old female patient with clinical features of velocardiofacial syndrome, which include facial dysmorphism, congenital heart disease (persistent truncus arteriosus and ostium secundum-type atrial septal defect), and a seizure syndrome. Array comparative genomic hybridization revealed a non-continous deletion spanning cytobands 2q31.1-to 2q31.3, confirming a diagnosis of 2q31 microdeletion syndrome. The patient has undergone supportive therapies for swallowing and speech. Additionally, we provide a review of the literature on previous cases to give context.ConclusionIn this report, we present the first documented case of a complex, discontinuous deletion spanning in the 2q31-2q32 regions. This case contributes to our understanding of the phenotypic and mutational spectrum observed in individuals with deletions in these cytobands. It underscores the significance of employing high-resolution techniques and comprenhensive analysis in diagnosing patients with complex phenotypes. Such approaches are crucial for differentiating this condition from more common microdeletion syndromes, such as the 22q11 deletion syndrome.

Effect of percutaneous closure on atrium and appendage functions in patients with secundum atrial septal defects.

The study aimed to assess the impact of percutaneous transcatheter atrial septal defect (ASD) closure on atrium and atrial appandage functions among patients with ostium secundum ASDs. A total of 101 patients (34,7% male, 65,3% female, 37,6 ± 12) with the diagnosis of ostium secundum type ASD underwent transthorasic (TTE) and transesophageal echocardiography (TEE) before and six months after percutaneous transcatheter ASD closure. Pulmonary venous flow and atrial appendage flow velocities were obtained from the TEE recordings. The offline evaluation of the global and segmental atrial appendage strains were evaluated with speckle tracking echocardiography (STE) via EchoPac 6,3 (GE Vingmed, Horten, Norway). Mean values of pulmonary artery pressure, right ventricular, left atrium, left ventricular end-diastolic and end-systolic diameters were significantly decreased 6 months after ASD closure. Statistically significant changes were documented in pulmonary venous and left atrial appendage flow velocities after ASD closure. Both left and right atrial appendage flow velocities and global strains of atrial appandages were improved after ASD closure. The mean left atrial appendage global strain value was - 11.45 ± 4.13% before the procedure, this value was - 16.82 ± 3.78% sixth months after the procedure (P < 0.001).The mean right atrial appendage global strain was - 13.31 ± 4.84% before the procedure and - 18.53 ± 4.69% sixth months after the procedure (P < 0.001). Left and right atrial appendage flow velocities and global strains of left and right atrial appandage can be improved after transcatheter ASD closure. Percutaneous transcatheter closure of ASDs not only improves atrial and left ventricular dimensions but also have a positive impact on left and right atrial appandage functions.

LATE HAEMOPHILLUS ENDOCARDITIS AFTER SEPTAL CLOSURE BY AMPLATZER

Abstract Introduction Atrial septal defects are the second most frequent congenital heart diseases and their treatment of choice is the percutaneous approach. Complications associated are infrequent and are mainly related with the puncture. Other complications such as embolism or endocarditis are uncommon. Clinical case A 17-year-old woman whose only medical history of interest was an ostium secundum-type atrial septal defect that was treated percutaneously implanting a 24-mm Amplatzer septal occlude. 15 months after the procedure she came to the emergency room presenting 40°C fever and a headache. Physical examination and tests results were normal, being discharged with antibiotic treatment. She returned 24 hours later due to persistence of the same symptoms. An echocardiogram was performed, revealing an endocarditic growth and uncomplete endothelization of the amplatzer. Following 10 days of antibiogram-guided therapy with good response, she underwent surgery. The device was extracted and the defect closed. PCR was positive for H. Parainfluenzae. The patient was discharged on the 7th day with antibiotic therapy. Discussion This case is relevant due to the late presentation of IE, incomplete endothelization and to being caused by infrequent IE germ source with a negative blood culture. Despite the low prevalence of these complications, it is necessary to rule them out through imaging tests as well as microbiological tests. Appropriate length of bacterial coverage usually extends from 6 months to 1 year after implantation. Documented cases of prosthetic IE related to incorrect endothelial coverage can lead to reassessing the time period during which prophylaxis should be maintained.

Atypical Atrial Tachycardia in a Newborn – a Case Report

Abstract We present the case of a neonatal patient with atrial tachycardia with unusual characteristics – abrupt onset and cessation, non-responsiveness to overdrive, termination by direct cardio-version – in association with an ostium secundum-type atrial septal defect and a large inter-atrial septum aneurysm. Electrocardiographic recordings showed P waves with characteristics suggesting a right low septal origin, which suggested a possible involvement of the septal aneurysm. In dealing with arrhythmias, a thorough echocardiographic evaluation is mandatory to identify underlying structural malformations and to evaluate the hemodynamic status. Further research should be made concerning mechanisms of arrhythmias in patients with an associated atrial septum aneurysm.

Holt-Oram Syndrome: A Variety of Heart-Hand Syndrome

Holt-Oram syndrome (HOS) is a heart-upper limb malformation complex , is a rare autosomal dominant disorder, caused by a mutation in the TBX5 gene located on chromosome 12 and near complete penetrance but variable expression. Holt and Oram first described this syndrome in 1960. Approximately 40% of cases represent new mutations. The syndrome is associated with defective development of cardiac structures that results in atrial septal defect(ASD), most commonly the secundum type; heart block of varying degree or both.The syndrome is characterized by cardiac malformations and aplasia or hypoplasia of the thumb. The incidence of HOS is estimated at 1:100,000 live births. In the literature, it is also known as atriodigital syndrome, heart-hand syndrome, upper limb-cardiovascular syndrome, cardiac-limb syndrome, or cardiomelic syndrome. Herein, we report a 45-year-old female case of HOS presenting a large ostium secundum type ASD along with congenitally fusion of carpal bones in both hands and absence of right first metacarpal bone with triphalagial thumb where as hypoplastic proximal phalange. In left hand, hypoplastic first metacarpal bone and hypoplastic thumb with syndactyly of both thumb with index finger.University Heart Journal Vol. 13, No. 2, July 2017; 67-71

Clinical characteristics and outcomes in pregnant women with Ebstein anomaly at the time of delivery in the USA: 2003–2012

Ebstein anomaly is an uncommon congenital cardiac lesion that may be associated with cyanosis, arrhythmias and right heart dysfunction. Investigation into patient characteristics and outcomes in pregnant women with Ebstein anomaly has been limited. To characterize patient characteristics and clinical events for pregnant women with Ebstein anomaly during hospitalization for delivery in the USA; also, to determine the effect of Ebstein anomaly on maternal clinical outcomes and individual predictors of poor outcome at time of delivery. We screened the Healthcare Cost and Utilization Project's National Inpatient Sample for hospital admissions of pregnant women for delivery (vaginal or caesarean section) in the USA from 2003-2012, and identified a cohort of 7,850,381. Clinical characteristics and maternal outcomes were identified in those with and without Ebstein anomaly. The primary outcome of interest was major adverse cardiac events (MACE), a composite of in-hospital death, acute myocardial infarction, cerebrovascular events, embolic events, cardiac complications of labour and delivery heart failure or arrhythmia. Our study population consisted of 82 hospitalizations of pregnant women with Ebstein anomaly and 7,850,299 without. The Ebstein cohort more frequently had ostium secundum-type atrial septal defect and/or patent foramen ovale and anomalous atrioventricular excitation (P<0.001 for both). The MACE rate was significantly higher among Ebstein patients (P<0.001). Preterm delivery, postpartum haemorrhage and caesarean delivery occurred more frequently among the Ebstein cohort (19.5% vs 7.2%, 8.5% vs 2.8% and 47.6% vs 31.1%, respectively; P≤0.001). In a multivariable analysis, anomalous atrioventricular excitation (odds ratio [OR] 21.75, 95% confidence interval [CI] 1.03-457.91) and preterm delivery (OR 11.71, 95% CI 1.39-98.89) were associated with MACE among those with Ebstein anomaly. Pregnant women with Ebstein anomaly are at higher risk of MACE during pregnancy and delivery. Preterm delivery occurred more frequently in women with Ebstein anomaly.

Total Anomalous Pulmonary Venous Connection in a Chicken

This report describes a case of total anomalous pulmonary venous connection (TAPVC) in a 5-wk-old male white leghorn chicken that presented with growth retardation. This chicken was a specific-pathogen-free chicken bred in an isolator. At 5 wk of age, the chicken was euthanatized and autopsied. Macroscopically, the right ventricle and right atrium were significantly enlarged whereas the left atrium was small and blind-ending with no connection to the pulmonary veins. The pulmonary veins were connected directly to the right atrium. The above abnormality was accompanied by an ostium secundum-type atrial septal defect. No other malformations were observed. TAPVC is a very rare congenital cardiac abnormality that has not been reported in avian species to date.

Prevalence, Mortality, and the Disease Burden of Pediatric Congenital Heart Disease in Taiwan

In Taiwan, the incidence of congenital heart diseases (CHDs) and severe CHDs was 13.08 and 1.51 per 1000 live births, respectively. This study further elucidates the prevalence and mortality of pediatric CHD patients in Taiwan. From the National Health Insurance database 2000-2010, we retrieved the data of CHD patients (aged 0-18 years). Mortality data were obtained from the national death statistics. In total, 45,119 pediatric CHD patients were identified, given the prevalence at 918.0 per 100,000 (107.1 for severe CHD and 853.8 for simple CHD). Ventricular septal defect, ostium secundum-type atrial septal defect, patent ductus arteriosus, pulmonary stenosis, and tetralogy of Fallot were the five most frequently diagnosed CHDs. In those aged 0-6 years, the prevalence was 1233.7 per 100,000 (123.5 for severe CHD and 1149.6 for simple CHD). The age-specific prevalence of both simple and severe CHDs declined rapidly after the age of 10 years. From the death registry, we noted that more than 90% of CHD-related deaths occurred before the age of 5 years. The probability of cardiac death in CHD patients during infancy was 4.5%, with the cumulative probability reaching 5.44%, 5.68%, and 6.04% by the ages of 5, 10, and 20 years, respectively. Because most CHD deaths occurred within the first 5 years of life (mainly during infancy), the relatively low prevalence of CHDs in the population aged 0-18 years (918/100,000; 74% for those between 0 years and 6 years of age) and the rapid decline in the age-specific prevalence of CHD after the age of 10 years was attributed to noncompliance of the children to medical follow-up after they began schooling.

Percutaneous transcatheter atrial septal defect closure with Amplatzer septal occluder device using three different techniques in three adult patients with complex ostium secundum type atrial defects

Complex ostium secundum type atrial septal defect (ASD) is a definition used for large (stretched diameter over 26 mm with deficient rim) or multiple ASDs or multi-fenestrated septum or ASDs with redundant and aneurysmal septal rims. Compared to simple defects, transcatheter ASD closure with Amplatzer septal occluder (ASO) is relatively challenging in these cases, and different techniques have been defined to increase procedure success. We report three adult patients with complex ostium secundum type ASDs that were closed with ASO device using three different techniques. The first case was a 36-year-old female patient with complaints of dyspnea, palpitation and fatigue. A complex ostium secundum type ASD was diagnosed, and the defect was closed with a 36 mm ASO device using a left upper pulmonary vein technique. The second case was a 47-year-old female patient with complaints of dyspnea and palpitation. A large and complex ASD was detected, and the defect was closed with a 28 mm device using a left atrial roof technique supported by non-inflated balloon. The third case was a 40-year-old female patient who presented with complaints of dyspnea and palpitation. Complex ASD was diagnosed, and the defect was closed with a 32 mm device using a partially inflated balloon technique. In large and complex ASDs, the classical implantation technique of an ASO device may fail at any time. The knowledge and application of different techniques that orient the left atrial disc parallel to the septum may increase the procedure success and decrease complications.

Index of Suspicion * Case 1: Irritability and Signs of Peritonitis in a Neonate * Case 2: Red Eye, Excessive Tearing, Photophobia, Irritability, and Splenomegaly in an Infant * Case 3: 2-year-old Girl Who Has Cough, Right Pulmonary Abnormalities

* ALL: : acute lymphoblastic leukemia CT: : computed tomography IVC: : inferior vena cava NA: : neonatal appendicitis NEC: : necrotizing enterocolitis WBC: : white blood cell A 7-day-old boy born at term is brought to the emergency department by his foster mother for refusal to feed for ∼20 hours and increased crying, although he is intermittently consolable. When given his bottle, the infant starts to suck but then cries as though in pain. He has not vomited, and his last bowel movement was yesterday. The stool has been moderately well formed without blood or mucus, and wet diapers have continued. There is no history of respiratory symptoms, lethargy, rashes, or jaundice. The biological mother is a 27-year-old woman with a known history of previous drug abuse; she had received some prenatal care. This infant was born by scheduled cesarean delivery. Initial physical examination reveals a crying but consolable neonate. His temperature is 37.7°C, heart rate is 170 beats/minute, and respiratory rate is 56 breaths/minute; the remainder of his findings are normal. His complete blood count (white blood cell [WBC] 11.7 × 109/L, 78.5% neutrophils, 11.4% monocytes, 9.8% lymphocytes), serum concentrations of electrolytes and glucose, urinalysis, and cerebrospinal fluid studies are normal. Cultures are obtained. An abdominal radiograph is normal and abdominal ultrasonography reveals trace pelvic fluid. He is given a bolus of normal saline and is started on antibiotics to treat possible sepsis. A few hours after admission, repeat examination is concerning for inconsolability, poor suck reflex, and lethargy. His abdomen is distended, firm, and tender to palpation. Bowel sounds are normal. Further studies are performed that reveal the diagnosis. A previously healthy 10-month-old boy is seen for a 2-week history of an erythematous left eye with tearing. Earlier, he was diagnosed as having conjunctivitis at an urgent care clinic and was treated with a 10-day course of polymyxin B/trimethoprim eye drops without improvement. He has felt warm intermittently but has not had a …

Interatrial shunt: diagnosis of patent foramen ovale and atrial septal defect with 64-row coronary computed tomography angiography

The aim of this study was to investigate the frequency with which interatrial shunts are found during routine coronary computed tomography (CT) angiography and to describe imaging characterizations of patent foramen ovale (PFO), atrial septal defect (ASD), and atrial septal aneurysm (ASA). A total of 1081 adult patients were evaluated retrospectively for interatrial shunting; 77 were excluded from the study. CT diagnosis of PFO was defined as (1) a channel-like appearance of the interatrial septum (IAS) and (2) a contrast agent jet flow from the left atrium (LA) to the right atrium (RA). ASD was defined as (1) the IAS resembling a membrane with a hole and (2) a contrast jet flow between the two atria. ASA was identified by detecting a minimum 10-mm protrusion of the LA beyond the IAS into the RA. Among 1004 patients, 86 patients (8.6%) were diagnosed to have PFO. Another 23 patients (2.3%) had a hole in the IAS and were diagnosed as having ASD: 21 with an ostium secundum-type ASD and 2 with the sinus venosus type. ASA accompanied ASD in three patients. Electrocardiography-gated CT using the saline-chaser contrast injection technique that is routinely used for coronary arterial imaging can be used to detect interatrial shunts. The technique can also serve as a method for differentiating PFO, ASD, and ASA.

Pulmonary Valve Infective Endocarditis in an Adult Patient with Severe Congenital Pulmonary Stenosis and Ostium Secundum Atrial Septal Defect

A hypertensive 76-year-old man with severe pulmonary valve stenosis (PVS) and recent initiation of haemodialysis was referred with fever, chills, and asthenia. One month prior, he had been admitted with similar symptoms. Transthoracic echocardiography (TTE) had shown a PVS and no valve vegetations were observed. Following discharge, he was readmitted with fever and blood cultures positive for Staphylococcus haemolyticus. A new TTE revealed two pulmonary valve vegetations and a previously undetected ostium secundum-type atrial septal defect (ASD), confirmed by transesophageal echocardiography. The clinical course was uneventful with intravenous antibiotic treatment and the patient was safely discharged. This is a case of pulmonary valve infective endocarditis (IE). The incidence of right-sided IE is on the rise due to the increased number of patients using central venous lines, pacing, haemodialysis and other intravascular devices. Pulmonary valve IE is extremely rare, especially in structurally normal hearts. The case reported here, presents a combination of predisposing factors, such as severe congenital PVS, the presence of a central venous catheter, and haemodialysis. The fact that it was an older patient with severe congenital PVS and associated with a previously undiagnosed ASD, is also an unusual feature of this case, making it even more interesting.

The utility of modified Butler-Leggett criteria for right ventricular hypertrophy in detection of clinically significant shunt ratio in ostium secundum–type atrial septal defect in adults

Background This study was performed to test the hypothesis that there exists a correlation between the Butler-Leggett (BL) criterion for right ventricular hypertrophy on the electrocardiogram and the Qp/Qs shunt ratio in adults with ostium secundum atrial septal defects (ASDs). Methods Demographic, cardiac catheterization, ASD closure, and electrocardiographic data were acquired on 70 patients with secundum ASDs closed percutaneously. Simple linear regression and logistic regression models were created to test the hypothesis. Results The mean Qp/Qs ratio and BL criterion value were 1.61 ± 0.46 and 0.11 ± 0.41, respectively. The BL criterion values correlated with shunt ratios ( r 2 = 0.11 and P = .004). A BL criterion value greater than 0 mV predicted a significant shunt ratio (Qp/Qs ≥1.5) (odds ratio, 4.8; 95% confidence interval, 1.3, 18.1; P = <.0001) with a sensitivity of 0.68 and specificity of 0.65. Conclusion Our results indicate that there is limited utility of the BL criterion at detecting right ventricular volume overload, although a BL criterion value greater than 0 mV being used to identify patients with significant intracardiac shunts yielded a sensitivity of 0.68 and specificity of 0.65.

Chylothorax Associated With Tricuspid Dysplasia and Atrial Septal Defect in a Bullmastiff

Transudate pleural effusion associated with tricuspid dysplasia and ostium secundum-type atrial septal defect was diagnosed in a 14-month-old bullmastiff. Following administration of furosemide and an angiotensin-converting enzyme (ACE) inhibitor, the dog remained free of pleural effusion for 10 months, until he showed severe dyspnea due to chylothorax. Medical therapy was unsuccessful to avoid recurrence of life-threatening pleural chylous effusion. Ligation of the thoracic duct and apposition of an omental pedicle flap were effective in the resolution of pleural chylous leakage.

Atrial Septal Defect: Anatomoechocardiographic Correlation

This study was undertaken to enhance understanding of the anatomic abnormalities involved in atrial septal defect (ASD) based on the anatomoechocardiographic comparison of equivalent specimens of ASD and the echocardiograms of patients with this anomaly. Of the 72 heart specimens, two had common atrioventricular canal (2.8%) and one had absence of right atrioventricular connection (1.4%). In all, 46 (63.8%) had fossa ovalis type ASD, two (2.8%) had true ostium secundum type ASD, two (2.8%) had ostium primum type ASD, two (2.8%) had superior sinus venosus type ASD, and two (2.8%) had inferior sinus venosus type ASD. One (1.4%) specimen had a coronary venous sinus type ASD and 14 (19.4%) had mixed type ASD. Of the 144 patients evaluated with echocardiography 15 (10.4%) had common atrioventricular canal, one (0.7%) had right absence of atrioventricular connection, 105 (72.9%) had ostium secundum type ASD (fossa ovalis), 10 (7%) had mixed type ASD, 9 (6.2%) had superior venous sinus type ASD, two (1.4%) had ostium primum type ASD, and two (1.4%) had true fossa ovalis type ASD. This series leads us to conclude that the key to successful management of ASD depends on understanding echocardiographic images in terms of anatomic specimens to provide appropriate evaluations for therapeutic decisions and establishment of prognoses.

HYPERTROPHIC CARDIOMYOPATHY WITH MIDVENTRICULAR OBSTRUCTION IN ASSOCIATION WITH OSTIUM SECUNDUM-TYPE ATRIAL SEPTAL DEFECT

This paper reports a 74 year-old-man with a hypertrophic cardiomyopathy associated with mid-ventricular obstruction and a secundum type atrial septal defect. He had been suffering from shortness of breath and occasional attacks of dizziness. Doppler echocardiography revealed the gradient in the mid-left ventricular area. A transesophageal echocardiogram confirmed the findings of transthoracic study and an ostium secundum-type atrial septal defect was documented. The present case represents a rare combination of hypertrophic cardiomyopathy associated with midventricular obstruction, and an ostium secundum type atrial septal defect in an elderly man.

Evaluation of P wave duration and P wave dispersion in adult patients with secundum atrial septal defect during normal sinus rhythm

Background: Paroxysmal atrial arrhythmias especially atrial fibrillation (AF) are frequently encountered in adult patients with atrial septal defect (ASD). Previously it was shown that maximum P wave duration and P wave dispersion in 12-lead surface electrocardiograms are significantly increased in individuals with a history of paroxysmal AF. The aim of this study was to determine whether P maximum and P dispersion in adult patients with ASD and without AF are increased as compared to healthy controls. In addition, the relationship of pulmonary to systemic flow ratio ( Qp/ Qs) and these P wave indices were investigated. Methods and results: Sixty-two consecutive patients [39 women, 23 men; mean age 33±13 years (range 16 to 61 years)] with ostium secundum type ASD and 47 healthy subjects [25 women, 22 men; mean age 36.6±9.5 years (range 18 to 50 years)] were investigated. P maximum, P minimum and P dispersion (maximum minus minimum P wave duration) were measured from the 12-lead surface ECG. There were no significant differences with respect to age ( P=0.08), gender ( P=0.3), heart rate ( P=0.3), left atrial diameter ( P=0.5) and left ventricular ejection fraction ( P=0.3) between patients and controls. Pulmonary artery peak systolic pressure was significantly higher in patients with ASD as compared to controls ( P<0.0001). P maximum was significantly longer in patients with ASD as compared to controls ( P<0.0001). In addition, P dispersion of the patients was significantly higher than controls ( P=0.001). P minimum was not different between groups ( P=0.12). Mean Qp/ Qs of the patients with ASD was 2.5±0.7 (minimum 1.5; maximum 4.1) and found to be significantly correlated with P maximum ( r=0.34; P=0.006) and P dispersion ( r=0.61; P<0.0001). Conclusions: Prolongation of P maximum and increased P dispersion could represent mechanical and electrical changes of atrial myocardium in patients with ASD. These changes of atrial myocardium may be more prominent with higher left to right shunt volumes.

Factores hemodinámicos y retraso de crecimiento en operados de comunicación interauricular

Ostium secundum-type atrial septal defect (ASD) is usually well tolerated, without severe complications in childhood. However, in many patients its effect on growth is disproproportionate when compared with that of more serious congenital heart disease. To test the hypothesis that hemodynamic disturbances are responsible for failure to thrive in congenital heart disease, we studied growth and the effect of surgical repair in children with ASD. The study was performed in 72 patients who underwent surgery at a mean age of 8 years and 8 months. The mean follow-up was 17 months. Somatometric and hemodynamic parameters were studied and the correlation between them was verified. The effect of surgery and of age at operation on somatometric changes was evaluated. Fifty-two percent of the patients were malnourished and height was affected in 28 %. The correlation between weight and age at operation was r 5 0.78 (p < 0.05) for the entire group, r 5 0.81 (p < 0.05) for the malnourished subgroup and r 5 0.88 (p < 0.05) for the subgroup with normal nutrition. No relationship was found between hemodynamic and somatometric parameters. Surgery improved growth disturbances but not in all patients (weight: from 52 % to 32 %; height: from 28 % to 16 %). Hemodynamic factors are not the only cause of growth and nutritional alterations.

Aortic coarctation, multiple ventricular septal defects, and anomalous coronary artery arising from the right pulmonary artery

The literature reveals no reports of a left coronary artery arising from the right pulmonary artery in association with aortic coarctation and multiple ventricular septal defects. The patient we describe here died of left myocardial infarction after palliative operation because this defect was unanticipated. Case report. The patient was admitted at the age of 12 days with a diagnosis of aortic coarctation and multiple ventricular septal defects. She had classic signs of aortic coarctation and predominantly left-sided heart failure, which responded partially to prostaglandin E1 and diuretics. Chest radiography revealed severe cardiomegaly and symmetrically plethoric lungs. The electrocardiogram showed sinus rhythm at 140 beats/min, right ventricular hypertrophy, and no signs of myocardial ischemia. Echocardiography demonstrated an aortic coarctation with long, severe hypoplasia of the arch, a large ductus arteriosus, and multiple ventricular septal defects. The left ventricle was neither hypokinetic nor hypoplastic. No coexisting mitral regurgitation was documented. There was also a left superior caval vein draining into the coronary sinus and a large ostium secundum–type atrial septal defect. The patient underwent pulmonary artery banding, division of the ductus arteriosus, and aortic coartectomy. Within 2 hours of the patient’s return to the intensive care unit, her condition deteriorated rapidly and dramatically, with a global but predominantly left-sided intractable heart failure, complete atrioventricular block, and cardiogenic shock. The postoperative electrocardiogram revealed signs of severe myocardial anterolateral ischemia and necrosis and a third-degree atrioventricular block (Fig. 1). Creatine kinase had increased to 530 U/L (normal range 15 to 100 U/L) with an MB fraction of 365 U/L (68%), representing a significant acute myocardial necrotic phenomenon. Postoperative cross-sectional echocardiography visualized a mildly dilated and severely hypokinetic left ventricle (less than 10% of shortening fraction) with severe impairment of diastolic function. The pulmonary artery banding was adequate, and there was no residual aortic coarctation. The right coronary artery was predominant, and the left coronary artery’s origin was not seen from the corresponding aortic Valsalva sinus. This raised suspicion of an anomalous left coronary artery arising from the pulmonary artery, which would provide an explanation for the myocardial infarction. Unfortunately, the child’s deteriorating condition did not allow performance of any further investigations. Despite maximum hemodynamic support and intravascular cardiac pacing with a 5F bipolar balloon pacing electrode (USCI International, BARD, C.R. Bard Ireland Ltd., Galway, Ireland), she died on the third postoperative day. Postmortem examination. The heart weighed 30 gm. There was, as expected, an abnormal left superior caval vein draining into the coronary sinus, a large ostium secundum–type atrial septal defect, and one large perimembranous and multiple muscular ventricular septal defects. The right ventricle was predominant, and the left one was small but not hypoplastic. Necrotic patches, confirmed by histologic analysis, were evident in the left anterolateral wall. The endocardium did not exhibit fibroelastosis. The aortic coarctation was well repaired, and the pulmonary banding was found to be in place. A tortuous, dilated right predominant coronary artery originated from the right aortic Valsalva sinus. The left coronary artery arose from the posterior right pulmonary artery wall immediately after the pulmonary bifurcation and followed a tortuous course over the anterior interventricular groove (Fig. 2). Discussion. Within the wide variety of left coronary anomalous origin, left coronary artery arising from the right pulmonary artery is extremely uncommon. The chief reason for this report, however, is the association of this origin with lesions producing high pulmonary flow, with consequent lack of clinical data suggesting such a diagnosis. Many factors led to misdiagnosis. First, this finding is not classically anticipated in cases of ventricular septal defects and aortic coarctation. Second, the left-to-right shunt provided normal perfusion to the aberrant coronary artery before the operation. Until palliation, this child’s left coronary blood flow was normal, and so were the left ventricular myocardial perfusion and oxygenation, because the pulmonary and aortic pressures and saturations were similar. The fall in pulmonary pressure to a low level after the pulmonary artery banding was therefore accompanied by a decline in the left coronary flow, with drops in coronary perfusion pressure and oxygen saturation leading to left ventricular ischemia and myocardial infarction. A similar physiopathologic condition related to closure of From the Department of Pediatric Cardiac Surgery, Centre Medico-Chirurgical de la Porte de Choisy, and the Department of Pediatric Cardiology, Institut de Puericulture de Paris, Paris, France.