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.

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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.

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.

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.