A term, healthy infant presented with respiratory distress and severe pulmonary hypertension (PH). With an unclear aetiology and the intent to decrease right ventricular afterload, pulmonary vasodilators were initiated. Follow-up imaging revealed a supravalvular mitral ring as the cause of the PH which resolved after surgical resection of the membrane. Supravalvular mitral ring is a rare left-side congenital heart disease lesion that can lead to PH usually between infancy and toddler years, but can be easily missed in the setting of a concurrent large atrial septal defect that masks the gradient across the left ventricular inflow. While bronchopulmonary dysplasia is the most common cause of PH in infancy, a thorough evaluation is critical for the diagnosis of this reversible cause of PH as a missed diagnosis may redirect care with pulmonary vasodilators in the acute setting and potentially worsen the clinical status.

Shone complex is a rare and underrecognized congenital heart disease involving four obstructive lesions of the left heart. This report presents a rare case of incomplete Shone complex, along with other associated anomalies, requires an extensive clinical workup for accurate diagnosis and appropriate surgical planning.

Shone complex (SC) is a rare multilevel congenital heart disease (CHD) characterized by four left-sided heart obstructive lesions: parachute mitral valve, supravalvular mitral ring, subaortic stenosis, and coarctation of the aorta (CoA), accounting for 0.6-0.7 % of CHD cases. A 4-week-old male neonate presented with severe respiratory distress, tachycardia (150 beats/min), tachypnea (40/min), and hypoxia (80 % saturation). Blood pressure was 90/55 mmHg in the upper arms; lower extremity measurements were challenging. ECG showed a heart rate of 150 beats/min, normal sinus rhythm, left axis deviation, and left ventricular (LV) hypertrophy. Transthoracic echocardiography revealed mild concentric LV hypertrophy and reduced ejection fraction (45 %). A supramitral ring led to severe supravalvular mitral stenosis, and a bicuspid aortic valve caused moderate aortic stenosis. Suprasternal views confirmed severe CoA distal to the left subclavian artery. The patient underwent successful CoA repair at five months, with ongoing surveillance for other defects. One year later, he remained stable with no significant pressure gradient changes. SC presents significant clinical challenge due to its associated congenital anomalies. Early echocardiographic diagnosis and timely surgical intervention are essential for optimizing patient outcomes, given the variability in severity and the potential for complications. Multidisciplinary management is crucial for addressing the complexities of this condition. This case illustrates effective staged surgical management of partial SC, emphasizing early diagnosis and the utility of point-of-care ultrasound.

Shone's syndrome (SS) is a rare congenital cardiac anomaly characterized by a spectrum of developmental abnormalities. It predominantly presents as consisting of a variety of left ventricular inflow and outflow tract lesions, with inflow tract lesions typically including parachute mitral valve and supravalvular mitral ring. However, reports of SS involving double-orifice mitral valve are scarce.

It is not uncommon for congenital heart defects to occur in clusters. Those involving a right to left heart shunt commonly cause cyanosis and finger clubbing. Differential clubbing involving only the lower limb digits is a strong pointer to the presence of patent ductus arteriosus with reversal of shunt. We report a case of 25-year-old man with effort intolerance and differential clubbing. He was found to have the uncommon triad of patent ductus arteriosus, ventricular septal defect and supravalvular ring mitral stenosis. The presence of differential clubbing on a background of patent ductus arteriosus usually indicates a reversal of shunt and negates surgical intervention. This general rule may however not apply with co-existing mitral stenosis as the elevated pulmonary pressure may be predominantly post-capillary. The finding of mitral stenosis in a patient with patent ductus arteriosus and differential limb clubbing may signify a good prognostic surgical outcome. Il n'est pas rare que des malformations cardiaques congénitales surviennent en clusters. Celles impliquant un shunt cardiaque droitegauche provoquent souvent une cyanose et un hippocratisme digital. L'hippocratisme digital différentiel touchant uniquement les orteils des membres inférieurs est un indicateur fort de la présence d'un canal artériel persistant avec inversion du shunt. Nous rapportons le cas d'un homme de 25 ans présentant une intolérance à l'effort et un hippocratisme digital différentiel. Il a été diagnostiqué avec la triade peu commune de canal artériel persistant, de communication interventriculaire et de sténose mitrale à anneau supravalvulaire. La présence d'un hippocratisme digital différentiel sur un fond de canal artériel persistant indique généralement une inversion du shunt et exclut une intervention chirurgicale. Cependant, cette règle générale peut ne pas s'appliquer en présence d'une sténose mitrale concomitante, car la pression pulmonaire élevée peut être principalement post-capillaire. La découverte d'une sténose mitrale chez un patient atteint de canal artériel persistant et d'un hippocratisme digital différentiel peut indiquer un bon pronostic pour l'intervention chirurgicale.