Double Inlet Left Ventricle with common AV valve, discordant ventriculo-arterial connection and pulmonary atresia: A rare anomaly on fetal echocardiogram with three dimensional spatio-temporal imaging correlation (STIC)

Double Inlet Left Ventricle through a common AV valve is a rare type of functionally univentricular heart. We report a fetus with double inlet left ventricle with discordant ventriculo-arterial connection and pulmonary atresia. The anatomy was readily apparent on three dimensional rendering by spatio-temporal imaging correlation technique(STIC)

Double Inlet Left Ventricle (DILV) with Mal-posed Great Vessels: A Challenge in a Developing World

Background: Double Inlet Left Ventricle (DILV) with transposition of the great vessels is rare in children with congenital heart disease. We present two cases seen at a paediatric echocardiography centre. Baby ND is a 28-day old female who presented with respiratory distress and cyanosis from the first day of life. Echocardiography showed a double inlet left ventricle and mal-posed great vessels. MB is a seven-month-old female who presented with breathlessness and cyanosis from the first day of life. Examination showed a soft pan-systolic murmur of grade 2 variety at the mid sternal boarder. ECG showed left ventricular dominance and abnormal T changes. Echocardiography revealed a double inlet left ventricle and mal-posed great vessels. Conclusion: Double inlet left ventricle and mal-posed great vessels is a rare complex cardiac anomaly of univentricular physiology. A high index of suspicion (especially if the new born presents with first day history of cyanosis and breathlessness) is necessary for quick referral and surgical intervention.

New Horizon of Intervention in Congenital Heart Disease: AFR in a Complex Cyanotic Patient

Double-inlet left ventricle (DILV) is the commonest form of the anatomic univentricular heart which has different ventriculoarterial connection; generally, the most prevalent type is DILV with the hypoplastic right ventricle on the left side. The disease is associated with several heart defects, and the treatment method is different based on the anatomy of the heart, but treatment methods are almost always palliative. In this paper, we described one adult patient with DILV and severe left AV (atrioventricular valve) stenosis who is managed with a novel palliative intervention; it means AFR (atrial flow regulator) device implantation for the first time.

Advanced therapies in complex congenital heart disease

Background Although there are some data on how to manage and treat patients with Eisenmenger syndrome due to simple cardiac defects, little evidence exists to guide best management of pulmonary vascular disease in cases with more complex anatomy such as common arterial supply to both the systemic and pulmonary circulations or univentricular physiology. Beyond the uncertainty on how to best assess and manage such patients, there are added concerns with regards to the potential effects of treatment on the risk of cardiac arrhythmia, valve regurgitation and excessive load to the ventricle with increased pulmonary blood flow. Case presentation We present a case of a patient with double inlet left ventricle and evidence of pulmonary hypertension and describe his outcome on the therapies prescribed. Conclusions We review the literature and describe our approach to the patient’s management in light of the available evidence and published literature.

Extracorporeal cardiopulmonary resuscitation with low pump flow for blocked modified Blalock–Taussig shunt followed by spontaneous recanalization

A 2-week-old male newborn with a double inlet left ventricle developed a cardiac arrest following modified Blalock–Taussig anastomosis in pediatric intensive care unit. Probable causes of the arrest were hemodynamic instability and thrombosed shunt, which was later recanalized on extracorporeal membrane oxygenation therapy, which was successfully used with a pump flow lower than recommended in these patients—without the shunt clip, but without any complications.

Failure of Cellularization of Ventriculotomy Patch Leading to Right Ventricular Pseudoaneurysm

Enlargement of the bulboventricular foramen (BVF) in double-inlet left ventricle or the ventricular septal defect (VSD) in tricuspid atresia with transposition of the great arteries is one approach for prevention or treatment of systemic ventricular outflow obstruction. Most often, BVF/VSD restriction is bypassed preemptively or addressed directly at the time of Glenn/Fontan procedures as part of staged univentricular palliation. We describe a patient who underwent enlargement of a restrictive VSD during Fontan completion and subsequently presented with an asymptomatic pseudoaneurysm of the right ventricle at the ventriculotomy site.