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A simplified technique of performing the extracardiac Fontan operation on cardiopulmonary bypass is described. The advantages of this technique are briefly discussed. [ABSTRACT FROM AUTHOR]

Total cavopulmonary connection (Fontan) without using cardiopulmonary bypass (CPB) may be superior to Fontan on CPB. In the experience of many, a Fontan operation without CPB may be associated with a reduced need for inotropic support, improved outcomes in the postoperative phase including faster time to termination of mechanical ventilatory support, reduced intensive care unit stay, lower volume of pleural and peritoneal effusions, and decreased hospital stay, thereby rendering it more cost-effective. However, the operation is technically more difficult to perform than Fontan on CPB and requires significant modifications of surgical technique and alteration in overall management strategy. In this article, an alternative technique of performing total cavopulmonary connection without CPB is described. Its advantages are briefly discussed. [ABSTRACT FROM AUTHOR]

Treating heart failure (HF) in postoperative congenital cardiac repair in children with an anomalous left coronary artery from the pulmonary artery (ALCAPA) is a daunting task. Regular anti-failure measures with angiotensin-converting enzyme (ACE) inhibitors may become inadequate or resistant in certain situations. Angiotensin receptor-neprilysin inhibitor (ARNI) in place of ACE inhibitors can be an alternative to help these postcardiac surgical children convalesce better. Sacubitril/valsartan is an ARNI-approved treatment for HF in adults, and in pediatric HF patients, it is showing promising benefits. However, in postcardiac surgical children with HF, its effects are not known. We used ARNI successfully in one of our postcardiac surgical infants with ALCAPA repair in HF, and we describe this case here. [ABSTRACT FROM AUTHOR]

In patients with univentricular heart, the Fontan procedure is the final palliation. This is usually staged. A systemic-to-pulmonary artery shunt is performed in the presence of episodes of cyanotic spells in the neonatal period or in some patients in infancy; a bidirectional superior cardiopulmonary anastomosis is preferred early in life. This is followed by the final Fontan palliation on an elective basis later. For an effective bidirectional superior cavopulmonary anastomosis and Fontan palliation, good-sized confluent pulmonary arteries (PAs) are mandatory in addition to favorable hemodynamic data. Patients with discontinuous PAs that are small in size present a surgical challenge at initial palliation as the one described in this report. [ABSTRACT FROM AUTHOR]

Total anomalous pulmonary venous connection (TAPVC) is a rare congenital heart disease with an incidence of 1%-3% of all congenital heart disease patients. The pulmonary veins returning oxygenated blood from the lungs are draining into the right side (right atrium or great veins), causing systemic hypoxia and cyanosis. TAPVC patients are mostly present at an early stage and need surgical correction to sustain life and prevent complications. However, some subsets of patients with TAPVC in whom the mixing of blood is happening may progress into adult life and have associated pulmonary arterial hypertension (PAH) and right ventricular (RV) dysfunction. We report a case of a young adult lady who presented to our hospital at 16 years of age with TAPVC along with severe PAH and RV dysfunction. During the perioperative period, the patient was successfully managed with pulmonary vasodilators and inotropic support. The objective of the case report was to describe the management strategy followed in the successful treatment of the patient. Due to congestive heart failure, low cardiac output status, and other factors, these individuals will also experience comorbidities such as PAH, RV dysfunction, and organ dysfunction. Patients require care in an environment that has experience handling a variety of problems and a PAH crisis. PAH can be controlled with the aid of pulmonary vasodilators such as sildenafil, bosentan, milrinone, NTG, and inhaled nitric oxide. Mechanical hyperventilation to lower pulmonary vascular resistance will also aid in treatment. When combined with intraoperative transesophageal echocardiography monitoring, the balanced anesthetic method can be used to successfully manage the majority of cases. [ABSTRACT FROM AUTHOR]

A 4.5-year-old patient with tricuspid atresia, pulmonary stenosis, bilateral superior vena cava veins with absent innominate vein, and hypoplasia of the left pulmonary artery required a bidirectional superior cavopulmonary anastomosis. An innominate vein was fashioned out of a 6 mm polytetrafluoroethylene graft. The technique is briefly discussed. [ABSTRACT FROM AUTHOR]

Paralysis of diaphragm on one or, exceptionally, both sides is a common cause of delayed recovery and excessive morbidity following pediatric cardiac surgery. The consequences of this complication after all forms of congenital heart surgery in newborns and young infants can be potentially serious. The impact of diaphragmatic palsy on the physiology after single ventricle palliations is particularly significant. It is necessary for all professionals taking care of children with heart disease to be familiar with the etiology, diagnosis, and management of this condition. Early recognition and prompt management of diaphragmatic palsy can potentially reduce the duration of mechanical ventilation and intensive care in those who develop this complication. This review summarizes the anatomy of the phrenic nerves, reasons behind the occurrence of diaphragmatic palsy, and suggests practical guidelines for management.

Persistent pleural effusions are a source of significant morbidity and mortality following surgery in congenital heart disease. In this review, we discuss the etiology, pathophysiology, and management of this common complication.

Pulmonary hypertension due to delay in presentation, diagnosis, referral, and surgery for septal defects is not uncommon in the developing world and translates into high morbidity and mortality following open heart surgery to close these defects. Leaving a small atrial communication may not always be effective. Extracorporeal membrane oxygenation and inhaled nitric oxide therapy in the immediate postoperative phase may not be available or may not be financially feasible in many institutions which are economically challenged. Unidirectional valved patch is emerging as a new and effective solution to this problem and promises to make at least the immediate postoperative results more predictable in this subset of patients.