Management of late presentation congenital heart disease.

In many parts of the world, mostly low- and middle-income countries, timely diagnosis and repair of congenital heart diseases (CHDs) is not feasible for a variety of reasons. In these regions, economic growth has enabled the development of cardiac units that manage patients with CHD presenting later than would be ideal, often after the window for early stabilisation - transposition of the great arteries, coarctation of the aorta - or for lower-risk surgery in infancy - left-to-right shunts or cyanotic conditions. As a result, patients may have suffered organ dysfunction, manifest signs of pulmonary vascular disease, or the sequelae of profound cyanosis and polycythaemia. Late presentation poses unique clinical and ethical challenges in decision making regarding operability or surgical candidacy, surgical strategy, and perioperative intensive care management.

Double outlet of both ventricles: morphological, echocardiographic and surgical considerations.

OBJECTIVES: To describe the morphology, echocardiographic features and surgical management of the entity appropriately described as 'double outlet of both ventricles'. METHODS: Seven patients (5 males, age 0.5-7.5 months) were diagnosed to have a unique form of subarterial ventricular septal defect (VSD) and ventriculo-arterial connection, where a muscular outlet septum straddled the crest of the ventricular septum in a cruciate manner, such that both great arteries were equally committed to both ventricles. Diagnosis was established by echocardiography, with 6 patients submitted to surgical repair by means of intracardiac routing of the left ventricle to the aorta using 2 patches. RESULTS: Surgical repair was successful in all 6 patients in whom it was attempted. In addition, 1 patient underwent concomitant repair of aortic coarctation, and 2 had closure of multiple VSDs. We lost 1 patient to follow-up after diagnosis. Follow-up with a range from 3 months to 8 years in the remaining patients revealed all to be clinically well with satisfactory growth of both outflow tracts. CONCLUSIONS: We describe a series of patients with the ventriculo-arterial connection best described as 'double outlet of both ventricles'. Diagnosis is readily established by echocardiography. Good early and midterm results can be expected subsequent to surgical repair using 2 patches for interventricular septation.

Large Right Ventricular Clot in Pulmonary Atresia With Intact Ventricular Septum: In Defense of Biventricular Approach.

Thrombus formation within the right ventricle (RV) in the setting of pulmonary atresia with intact ventricular septum (PAIVS) is not a very common occurrence and can be catastrophic. We present the case of a seven-month-old child with PAIVS and RV clot who successfully underwent biventricular repair. We discuss the interesting case and the rationale for management by means of biventricular repair over single ventricle repair when feasible in such a setting.

Calcified ductal aneurysm with severe aortic regurgitation.

A rare case of calcified aneurysm of the ductus arteriosus with severe aortic regurgitation is presented. We believe this is the first report of such a case in the English literature.

Management Issues in Intensive Care Units for Infants and Children with Heart Disease.

Admission of infants and children with cardiac disease to the neonatal (NICU) and pediatric ICU (PICU) is ever increasing in India (30-50 % of all admissions). The commonest indication for admission to the NICU or PICU is acute deterioration of cardiac disease. This includes: acute heart failure, hypercyanotic spells, arrhythmias, pericardial tamponade and sick cardiac neonates who need urgent intervention. Other increasingly frequent indications for ICU admission include heart failure with concomitant chest infection and impending respiratory failure and, severe cyanotic heart disease with various stroke syndromes. It is thus essential that a pediatrician be comfortable with the ICU management of such children and that low cost ICU modalities be utilized in order to reach out to as many children as feasible. It is heartening that there is renewed interest in inexpensive therapies like noninvasive ventilation and therapeutic hypothermia.

Nutritional support in the critically ill child.

The nutritional management of a critically ill child is not a glamorous subject and seldom receives the attention that it merits despite increasing evidence that appropriate goal-oriented nutritional support is associated with improved outcome. Current nutritional management is based on rapidly emerging knowledge on the very special nutritional requirements related to the "vastly different metabolic and physiologic characteristics of the hypermetabolic and stressed" critically ill child. There has been significant changes in traditional practice particularly in the area of calorie delivery, amount of macronutrients and route of nutrient delivery in the critically ill child. The critically ill child presents with "greatly disordered nutrient metabolism" and successful nutritional support involves an initial "hypocaloric regime" with a precise mix of carbohydrates, proteins and lipids which need periodic review as the child improves. The therapeutic benefits of minimal enteral feeding (MEF) have been clearly established--MEF being associated with diminished morbidity, infection rates as well as reduced ICU and hospital stays. Immune enhancement has also been shown to be of some benefit in the critically ill but the subject needs further study.

Management of perioperative low cardiac output state without extracorporeal life support: What is feasible?

A transient and reversible reduction in cardiac output-low cardiac output state (LCOS) often occurs following surgery for congenital heart disease. Inappropriately managed LCOS is a risk factor for increased morbidity and death. LCOS may occasionally be progressive and refractory needing a period of "myocardial rest" with extracorporeal life support (ECLS). ECLS is currently considered a routine tool available for rapid deployment in most industrialized countries. Accumulated experience and refinements in technology have led to improving survivals - discharge survivals of 35%-50%, with almost 100% survival in select groups on elective left ventricular assist device. Thus, there is an increasing trend to initiate ECLS "early or electively in the operating room" in high-risk patients. India has a huge potential need for ECLS given the large number of infants presenting late with preexisting ventricular dysfunction or in circulatory collapse. ECLS is an expensive and resource consuming treatment modality and is not a viable therapeutic option in our country. The purpose of this paper is to reiterate an anticipatory, proactive approach to LCOS: (1) methods for early detection of evolving LCOS and (2) timely initiation of individualized therapy. This paper also explores what is feasible with the refinement of "simple, conventional, inexpensive strategies" for the management of LCOS. Therapy for LCOS should be multimodal based on the type of circulation and physiology. Our approach to LCOS includes: (1) intraoperative strategies, (2) aggressive afterload reduction, (3) lusitropy, (4) exclusion of structural defects, (5) harnessing cardiopulmonary interactions, and (6) addressing metabolic and endocrine abnormalities. We have achieved a discharge survival rate of greater than 97% with these simple methods.