Malposition of septum primum in isolated dextrocardia: unique and rare form of anomalous pulmonary venous return in association with partial absence of pericardium-case report

Background Total anomalous pulmonary venous return (TAPVR) refers to an anomaly in which all of the pulmonary veins drain directly or indirectly to the systemic venous circulation. However, unusual types constitute approximately 5% or less of TAPVRs and there may be obstruction or discontinuity of pulmonary vein at various levels. Case presentation A 3-month-old infant was presented to us with history of poor feeding, respiratory distress and desaturations. The routine echocardiographic investigation initially confirmed the diagnosis of an atrial septal defect with dextrocardia. However, due to disproportionate severity of symptoms and congestive heart failure a cardiac computer tomography angiogram was done that revealed a rare finding of connection of pulmonary veins fused with the posterior atrium, but on the rightward side of the deviated atrial septum. Therefore, pulmonary veins entered a sinus that drains directly into the right atrial superior-posterior wall. During surgical repair, we found an area of absent pericardium in the diaphragmatic surface of the heart. The patient underwent total repair of the TAPVR and patch reconstruction of the pericardial defect. The patient is doing well at 6-month follow up. Conclusions The septum primum malposition defect resulting in TAPVR is a very rare congenital anomaly that can be rarely seen without any heterotaxy. The anomalous features including absent pericardium and dextrocardia were present in this patient have not been described previously with TAPVR. Therefore, we have hypothesized the embryological correlation of absent pericardium and cardiac malposition in such case. Transthoracic echocardiography with Doppler interrogation is a reliable method for diagnosing this condition. In case of suboptimal echocardiographic image due to cardiac position, unclear anatomy or unexplained symptoms, advanced imaging such as computer tomographic angiography or cardiac magnetic resonance imaging can be very helpful. Preoperative proper diagnosis of this anomaly facilitates successful surgical management with excellent outcome.

Adult congenital diaphragmatic hernia with absent pericardium and liver heterotopia

Adult congenital diaphragmatic hernia (CDH) with an absent pericardium and liver heterotopia is extremely rare, and had only been described in 1 patient. Here, we present another case of left-sided central CDH in a 40-year-old Chinese woman.

Absent pericardium: at risk for endocarditis?

A 16-year-old girl with no previous cardiac problems presented to the casualty with septic shock. Investigations revealed endocarditis involving the mitral valve. At the time of surgery, she was noted to have a large left atrial appendage herniating into the left pleural cavity due to partial absence of the pericardium. Complete or partial absence of the pericardium is an uncommon congenital anomaly. Though various complications and presentations are reported, it has not been identified as a risk factor for endocarditis. We discuss the possible mechanism, and need for prophylaxis against endocarditis, in patients with absence of the pericardium.

Pentalogy of Cantrell: A Case Report with Pathologic Findings

We present the case of a 28-h-old female infant born at 37 weeks of gestation with a rare congenital malformation consisting of a pentad of findings: ectopia cordis, a midline supraumbilical wall defect, a defect of the lower sternum, absent pericardium, and an anterior diaphragmatic defect. This constellation of defects is known as the pentalogy of Cantrell. Additional autopsy findings included a bilateral cleft lip and palate, bilateral pulmonary hypoplasia, an atrial septal defect, and a patent ductus arteriosus. We present this case because of its rarity and discuss the pathologic findings.

Influence of pericardial constraint on atrioventricular interactions

The effects of the pericardial constraint in control, tamponade, and absent pericardium conditions was studied in 18 anesthetized open-chest dogs. Atrial pressures and systolic and diastolic inflow volumes per beat in the superior (SVC), inferior vena cavae (IVC), and pulmonary vein (PV) were measured. With increasing tamponade, 1) the systolic-diastolic distribution of venous flow became almost exclusively systolic in the SVC (P less than 0.001) and IVC (P less than 0.05), as the gamma-descent disappeared; 2) similar but lesser left-sided changes occurred in PV flows (P less than 0.001) and pressure; and 3) the systolic-diastolic distribution of venous flow was modulated by heart rate. The results imply that during tamponade 1) increased pericardial liquid pressure associated with an increased pericardial constraint couples reciprocal atrial and ventricular volume changes; 2) the atria fill during ventricular ejection (atrioventricular interaction); 3) total heart volume must have been relatively constant throughout a cardiac cycle; and 4) differences in right and left heart compliances may explain persistent diastolic PV flow. Pericardiotomy produced a small increase in the ratio of diastolic to systolic venous inflow volumes (P less than 0.025), suggesting that normally the pericardium has a minor influence on atrioventricular interaction, the volume changes in the atria and ventricles being relatively uncoupled. With severe tamponade, a homogeneous pericardial fluid column tightly couples atrial and ventricular volume changes and accounts for the characteristic changes in atrial pressure waveforms and patterns of venous flow.