Current perspective of immunomodulators for lung transplant.

Lung transplantation is an effective treatment option for selected patients suffering from end-stage lung disease. More intensive immunosuppression is enforced after lung transplants owing to a greater risk of rejection than after any other solid organ transplants. The commencing of lung transplantation in the modern era was in 1983 when the Toronto Lung Transplant Group executed the first successful lung transplant. A total of 43,785 lung transplants and 1365 heart-lung transplants have been performed from 1 Jan 1988 until 31 Jan 2021. The aim of this review article is to discuss the existing immunosuppressive strategies and emerging agents to prevent acute and chronic rejection in lung transplantation.

Scaling skin and failing heart: the cardio-cutaneous connection.

Ichthyosis refers to a comparatively rare group of skin disorders which may present with associated cardiomyopathy. We report a case of an 11-year-old female child who presented with ichthyosis and associated dilated cardiomyopathy. Genetic testing revealed mutation in the RBCK1 gene. She was successfully managed with heart transplantation. The purpose of the case report is to embark on the association between the skin and heart, the role of desmosomes, and the cutaneous manifestations of life-threatening cardiac disease. Cutaneous manifestations should not be escaped, as some of which could be a marker for sudden cardiac death and appropriate corrective actions can potentially save life.

Combined Warden and Mustard procedure for late presenting transposition with anomalous pulmonary venous connections and regressed left ventricle.

The presence of systemic and pulmonary venous anomalies in late presenting transposition of great arteries (TGA) may make standard atrial switch procedures difficult, necessitating modification in the surgical procedure. We present a case report of a 13-year-old boy with a partial anomalous connection of right superior pulmonary vein with TGA and regressed left ventricle, where a combination of Warden procedure and Mustard procedure was used.

Arterial switch for double-outlet left ventricle - Diagnostic and surgical considerations.

Double-outlet left ventricle (DOLV) is a rare congenital cardiac anomaly in which both the aorta and pulmonary artery arise completely or predominantly from the left ventricle. DOLV is a spectrum and can be classified depending on the position of the ventricular septal defect (VSD) relative to the great vessels, the relationship of the great vessels, and the presence or absence of pulmonary or aortic outflow obstruction. In the absence of tricuspid atresia or hypoplastic right ventricle, two ventricle repair is the preferred surgical treatment. We report a 31-day-old, 2.1 kg neonate with DOLV, subaortic VSD who underwent a successful arterial switch with VSD closure.

Heart transplantation for patients with single ventricle physiology.

BACKGROUND: There is a growing population of palliated and unpalliated single ventricle physiology patients for whom heart transplantation is the only treatment option available. There is a paucity of reports of heart transplantation in this challenging and growing subset of patients from our part of the world. The purpose of the article is to briefly review our experience in the subgroup and compare it with the available literature. METHODS: This was a single-institution retrospective observational study of 16 patients with single ventricle physiology who were transplanted between 2016 and 2019 and their outcomes. The study groups were divided into those with ventricular dysfunction (group 1), who fare substantially better than those with normal ventricular function (group 2) whose short-term outcomes were poorer. Worsening cyanosis, poor candidature for completion Fontan procedure due to severe atrioventricular valve regurgitation or pulmonary artery anatomy, protein-losing enteropathy, plastic bronchitis, and worsening systemic venous congestion are indications in those with normal ventricular function. RESULTS: Patients with ventricular dysfunction as the main indication had excellent early survival with no early mortality compared to 40% mortality in patients with normal ventricular function. Patients who survived to leave the hospital had however similar long-term outcomes. Two patients with protein-losing enteropathy resolved completely by one month. Normal ventricular function, pulmonary artery stenting, early Fontan failure (6 months), ascites, and need for desensitization were risk factors for early mortality. After the early acute phase of increased risk, the mortality risk plateaued off. CONCLUSION: Transplantation in patients with single ventricle and ventricular dysfunction can be offered with a good early and late outcome. There is a need to have multi-institutional and multi-disciplinary collaboration along with work in basic sciences to better understand the effects of failed Fontan physiology with normal ventricular function.