Kawasaki disease (KD) is now a common cause of acquired heart disease in children. Coronary artery involvement is the most serious complication in children with KD. Several non-coronary complications have now been identified in this condition but these are often overlooked. Myocarditis is an integral component of KD and may be more common than coronary artery abnormalities. Pericardial involvement and valvular abnormalities have also been observed in patients with KD. KD shock syndrome is now being increasingly recognized and may be difficult to differentiate clinically from toxic shock syndrome. Endothelial dysfunction has been reported both during acute stage and also on follow-up. This may be a potentially modifiable cardiovascular risk factor.

Multisystem inflammatory syndrome in children (MIC-S) is a hyperinflammatory manifestation of severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) infection. Information on the long-term outcome of MIS-C is limited. This study was conducted to describe the long-term outcome of MIS-C from a tertiary care center in North India. Children admitted with MIS-C from September 2020 to January 2021 were followed up after discharge until June 2021. The details during the acute phase (clinical features, investigations, treatment, and outcome) and follow-up (symptoms, echocardiographic findings, ongoing treatment, and outcome) were collected retrospectively. During the acute phase, 40 children presented at median (interquartile range [IQR]) age of 7 (5–10) years with fever, mucocutaneous, gastrointestinal, and respiratory symptoms. The majority (66.7%) of the children had positive SARS-CoV-2 serology and elevated inflammatory markers (C-reactive protein, procalcitonin, ferritin, D-dimer, and fibrinogen), lymphopenia, and thrombocytopenia. Eighty percent had shock, 72.5% had myocardial dysfunction (left ventricular ejection fraction <55%), and 22.5% had coronary artery dilatation or aneurysm. Treatment included pediatric intensive care unit admission (85%), intravenous immunoglobulin (100%), steroids (85%), aspirin (80%), vasoactive drugs (72.5%), and invasive mechanical ventilation (22.5%). Two (5%) children died because of refractory shock. Thirty-four children were followed up with until a median (IQR) of 5 (3–6) months. During the follow-up, a majority were asymptomatic, myocardial function returned to normal in all, and only one had coronary artery aneurysm. Prednisolone and aspirin were given for a median (IQR) of 3 (2–4) weeks and 4 (4–6) weeks after discharge, respectively. There was one readmission and no death during the follow-up. To conclude, the long-term outcome of MIS-C is generally favorable with resolution of cardiovascular manifestations (myocardial dysfunction and coronary artery changes) in the majority of children during follow-up.

Background Mucormycosis is an invasive fungal infection (IFI) most commonly seen in immunocompromised patients. Diabetic ketoacidosis, haematopoietic transplantation, iron overload states, and deferoxamine therapy are considered to be some of the classical risk factors. While cutaneous and rhino-sinusoidal forms may be seen in immunocompetent (IC) individuals, cardiac and mediastinal involvement is rare. In this report, we describe a young patient without predisposing factors who presented as mediastinal mucormycosis with extensive cardiac involvement. Case summary A 19-year-old male presented with complaints of dry cough and dyspnoea on exertion over the last 4 months. Echocardiography showed diffuse infiltration of both atria along with multiple pedunculated freely mobile masses. A computed tomography chest was done to further delineate the true extent of the disease and revealed diffuse infiltration of the mediastinum, bilateral atria and interatrial septum, pulmonary veins, and superior vena cava. A fine needle aspiration cytology from a mediastinal mass revealed broad aseptate fungal hyphae with right angled branching consistent with Mucor. Extensive evaluation could not find any predisposing factors. The patient was started on Amphotericin B and surgical debridement was contemplated. However, owing to the diffuse infiltration around the heart and mediastinal vasculature, debridement could not be performed and the patient eventually succumbed to the illness. Discussion Mediastinal mucormycosis though rare in IC patients, is a rapidly progressive condition with a high fatality. A high index of suspicion needs to be maintained in individuals presenting with infiltrative disorders of the mediastinum for early diagnosis and prompt treatment.

Internal thoracic artery aneurysms (ITAAs) are rare with wide variation in clinical presentation and a high risk of rupture. Endovascular techniques are increasingly being used for treatment of such aneurysms over surgical repair in recent times. A 34-year-old male presented with progressive swelling of the right anterior chest wall for 2 weeks and was diagnosed with right internal thoracic artery aneurysm with contained rupture. He underwent successful endovascular repair with coil embolization of ruptured right ITAA. Post intervention computed tomography (CT) angiography confirmed sealing of the ruptured aneurysm with no residual filling of the sac. At six months follow-up he is doing well with complete resolution of hematoma. This case demonstrates that an endovascular approach with coil embolization is a feasible and safe option for treating the rare ruptured ITAAs.

Background Pulmonary artery thrombosis is rare in neonates and mimics as persistent pulmonary hypertension or congenital heart disease. Risk factors include septicemia, dehydration, polycythemia, maternal diabetes, asphyxia, and inherited thrombophilias. They present with cyanosis and respiratory distress. Careful echocardiogram assessment helps in identifying the thrombus in the pulmonary artery and its branches. Computed tomography pulmonary angiography confirms the diagnosis. Case presentation We present a case of term neonate who presented with respiratory distress and cyanosis and a detailed echocardiogram revealed thrombus in the origin of left pulmonary artery. The neonate was managed initially with unfractionated heparin and later with low molecular weight heparin with which there was significant resolution of the thrombus Conclusion Spontaneous pulmonary artery thrombosis though rare should be suspected in any cyanotic neonate with respiratory distress. Management in these cases depends on the haemodynamic instability and lung ischemia.

Background Hypoplastic right heart syndrome with pulmonary atresia is a rare cyanotic heart disease with poor prognosis requiring urgent intervention to establish the pulmonary blood flow. Pulmonary blood flow is achieved by BT shunt or percutaneous techniques like PDA stenting or pulmonary valve perforation. Various series have shown that early surgical intervention causes high mortality in these patients. Pulmonary valve perforation is a suitable, physiological alternative to surgical techniques in selected patients. Case presentation We report a case of hypoplastic right heart syndrome with pulmonary atresia and restrictive VSD presenting with cyanosis from birth and underwent pulmonary valve perforation successfully. Conclusion Duct-dependent pulmonary circulation is a pediatric emergency, palliative procedure for establishing adequate pulmonary blood flow is essential early in the life. In the management of duct-dependent pulmonary circulation, RVOT perforation is an effective and safe option in suitable high-risk subgroups. The induced pulmonary regurgitation along with established physiological antegrade flow would be beneficial in the remodeling of tripartite/hypertrophied small RV.

Truncus arteriosus (common arterial trunk) is known to be associated with interrupted aortic arch in 10% to 15% of cases. However, the association of either of these lesions with a hypoplastic left ventricle is rare. The combination of all three of these lesions along with an intact interventricular septum is virtually unknown with only two cases reported in the literature. We report such a case with its anatomical and surgical management aspects.

No abstract