Case report: the 'vanished' left pulmonary artery.

Background: We report a case of isolated ductal origin of pulmonary artery (DOPA) diagnosed in an asymptomatic newborn. The primary aim of this case is to highlight the need to investigate for DOPA in patients diagnosed with an 'absent branch pulmonary artery'. Case summary: Our patient was an asymptomatic newborn infant, with normal intracardiac anatomy. He was initially diagnosed post-natally with 'absent left pulmonary artery' (LPA), though the LPA was seen in antenatal scans. He underwent angiography and was re-diagnosed with bilateral arterial ducts, with ductal origin of the LPA from the left arterial duct. The LPA was salvaged by first stenting the left arterial duct on Day 11 of life, with subsequent surgery to connect the LPA to the main pulmonary artery at 4.5 months old. The patient had an uneventful recovery after the surgery. Discussion: Ductal origin of pulmonary artery is a rare vascular anomaly characterized by continuity of the left or right pulmonary artery (PA) with the distal end of the arterial duct, and discontinuity with the main PA. It is commonly misdiagnosed as pulmonary artery agenesis when the patent arterial duct constricts, with cessation of blood flow into the affected pulmonary artery. A high index of suspicion is necessary for diagnosis of DOPA. Once diagnosed, this lesion is clearly amenable to intervention, with benefits from unifocalization, to prevent late onset pulmonary hypertension or cardiac failure.

Endocardial Fibroelastosis as an Independent Predictor of Atrioventricular Valve Rupture in Maternal Autoimmune Antibody Exposed Fetus: A Systematic Review with Clinicopathologic Analysis.

BACKGROUND: Neonatal lupus (NL) is a clinical syndrome that develops in the fetus as a result of maternal autoimmune antibodies. Congenital complete heart block (CHB) is the most common manifestation, while extranodal cardiac manifestations of NL, such as endocardial fibroelastosis (EFE) and myocarditis, are rare but more serious. Less is known about this atrioventricular valve rupture due to valvulitis as a consequence of maternal autoantibodies. We have described a case of cardiac neonatal lupus with an antenatally detected CHB patient who developed mitral and tricuspid valve chordal rupture at 45 days of age. We compared the cardiac histopathology and the fetal cardiac echocardiographic findings of this case with another fetus that was aborted after being antenatally diagnosed with CHB but without valvar rupture. A narrative analysis after a systematic review of the literature regarding atrioventricular valve apparatus rupture due to autoimmune etiology along with maternal characteristics, presentation, treatment, and outcome have been discussed in this article. OBJECTIVES: To describe published data on atrioventricular valve rupture in neonatal lupus, including clinical presentation, diagnostic evaluation, management, and outcomes. METHODS: We conducted a PRISMA-compliant descriptive systematic examination of case reports that included accounts of lupus during pregnancy or in the newborn period that resulted in an atrioventricular valve rupture. We gathered information on the patient's demographics, the details of the valve rupture and other comorbidities, the maternal therapy, the clinical course, and the results. We also used a standardized method to evaluate the cases' quality. A total of 12 cases were investigated, with 11 cases drawn from 10 case reports or case series and 1 from our own experience. RESULTS: Tricuspid valve rupture (50%) is more common than mitral valve rupture (17%). Unlike mitral valve rupture, which occurs postnatally, the timing of tricuspid valve rupture is perinatal. A total of 33% of the patients had concomitant complete heart block, while 75% of the patients had endocardial fibroelastosis on an antenatal ultrasound. Antenatal changes pertaining to endocardial fibroelastosis can be seen as early as 19 weeks of gestation. Patients with both valve ruptures generally have a poor prognosis, especially if they occur at close intervals. CONCLUSION: Atrioventricular valve rupture in neonatal lupus is rare. A majority of patients with valve rupture had antenatally detected endocardial fibroelastosis in the valvar apparatus. Appropriate and expedited surgical repair of ruptured atrioventricular valves is feasible and has a low mortality risk. Rupture of both atrioventricular valves occurring at close intervals carries a high mortality risk.

Saline contrast echocardiography complements cardiac interventions in neonates with transposition of great arteries and abnormal ductus venosus anatomy.

We present a rare case of premature low birthweight neonate with right diaphragmatic hernia and transposition of great vessels requiring balloon atrial septostomy. Congenital diaphragmatic hernia poses a unique challenge to umbilical venous catheterisation. Based on the radiographic position of umbilical vein catheter, umbilical venous cannulation was attempted; however, the catheter could not be navigated to the right atrium. Saline contrast echocardiography was used to delineate the abnormal umbilical and ductus venosus drainage. Eventually, the procedure was successfully completed via the femoral venous approach. We emphasise the importance of defining ductus venosus anatomy and umbilical venous drainage using a simple tool like saline contrast echocardiography before performing catheterisation using the umbilical venous access in such cases.

Absent pulmonary valve syndrome coexisting with coarctation of aorta in an adult.

The combination of absent pulmonary valve syndrome with coarctation of aorta is extremely uncommon and pathogenetically intriguing. This unusual association, which defies popular theories of pathogenesis of coarctation, may need to be looked at in the light of the current concepts regarding the morphogenesis of ventricular outflow tracts and aortic arch and the role of neural crest cells in both. A 19-year-old man with this combination underwent single-stage surgical correction though median sternotomy. The surgical management of this condition in adulthood has not been reported before.

Visceral heterotaxy in the developing world.

BACKGROUND: The challenge of visceral heterotaxy (VH) in the developing world has not been analysed in detail. METHOD: Retrospective chart review of 69 consecutive patients over ten years assessed the clinical profile and surgical outcome of VH. Median age: 3 years; median weight: 15kg. Diagnosis was made by echocardiography supplemented with blood smear (Howell Jolly bodies), Multi-Detector Computed Tomography (MDCT) angiogram and/or surgical inspection. RESULTS: In right isomerism (RI) group (n=32), 12 patients did not undergo surgery, five had Blalock Taussig shunt, 14 had bidirectional Glenn and one had Fontan completion, with surgical mortality of 5%. In left isomerism (LI) group (n=31), 11 patients underwent two ventricle repair (35%) and 15 (48%) had single ventricle repair, with surgical mortality of 3.8%; five did not have surgery. On follow up (median period 1.5 years), 33% of un-operated patients and 25% of operated patients died, mortality being higher for RI patients. Late mortality was due to sepsis, heart failure or arrhythmia. CONCLUSION: VH can be diagnosed by imaging based criteria. VH tends to present late in the developing world with a significant percentage inoperable. LI had better surgical outcome and higher long term survival.

Scimitar syndrome with right hemianomalous pulmonary venous drainage into superior vena cava/right atrium junction.

Scimitar syndrome is characterized by anomalous pulmonary venous drainage of a part or the whole of the right lung into the inferior vena cava (IVC). It is extremely uncommon in scimitar syndrome for the pulmonary veins to drain anomalously to a site other than the IVC. The following case report describes an otherwise classical scimitar syndrome with anomalous drainage of the right lung cephalad into the right atrium near its junction with the superior vena cava.

Computed tomographic angiography in tetralogy of Fallot.

Echocardiography is often inadequate for imaging tetralogy of Fallot, prompting cineangiography. This study prospectively evaluated multidetector computed tomographic angiography for preoperative evaluation of tetralogy of Fallot in 112 consecutive patients. Forty-eight had nonconfluent or hypoplastic pulmonary arteries (mean z-score, -2; range, -11.1-0.13) permitting only palliative or no surgery; 64 had adequate pulmonary artery anatomy (mean z-score, 0.59; range, -2.53-3.4) allowing total repair. The surgical data of 50 patients who underwent total correction were compared with transthoracic echocardiography and multidetector computed tomographic angiography findings. Multidetector computed tomographic angiography tended to reveal unsuspected collaterals and coronary abnormalities besides outlining the right ventricular outflow tract and pulmonary artery branches. The branch pulmonary artery diameter z-score was the most important determinant of surgical strategy, with the worst figures being associated with no surgical options or palliative surgery, and the best figures leading to corrective surgery. The mean radiation dose was 3.45 mSv. Multidetector computed tomographic angiography is a powerful supplement to echocardiography in the preoperative evaluation of tetralogy of Fallot.