Margarita Brida

Body mass index in adult congenital heart disease

Objective Abnormal body mass index (BMI) is associated with higher mortality in various cardiovascular cohorts. The prognostic implications of BMI in adults with congenital heart disease (ACHD) are unknown. We aim to assess the distribution of BMI and its association with symptoms and survival in the ACHD population. Methods We included 3069 ACHD patients (median age 32.6 years) under follow-up at our institution between 2001 and 2015. Patients were classified based on BMI as underweight (<18.5), normal weight (18.5–25), overweight (25–30) or obese (>30), and symptoms, exercise capacity and mortality were assessed. Results Overall, 6.2% of patients were underweight, 51.1% had normal weight, 28.2% were overweight and 14.6% were obese. Higher BMI values were associated with lower all-cause and cardiac mortality on univariable Cox analysis, and this effect persisted after adjustment for age, defect complexity, cyanosis and objective exercise capacity. Higher BMI was especially associated with better prognosis in symptomatic ACHD patients (HR 0.94 (95% CI 0.90 to 0.98), p=0.002) and those with complex underlying cardiac defects (HR 0.96 (95% CI 0.91 to 0.997), p=0.048) In patients with a complex cardiac defect who had repeated weight measurements, weight loss was also associated with a worse survival (HR 1.82 (95% CI 1.02 to 3.24), p=0.04). Conclusions ACHD patients with a higher BMI had a lower mortality. The association between BMI and mortality was especially pronounced in symptomatic patients with complex underlying cardiac defects, suggesting that cardiac cachexia may play a role. Indeed, weight loss in complex ACHD patients was linked to an even higher mortality.

Early mortality and concomitant procedures related to Fontan conversion: Quantitative analysis

BACKGROUND The Fontan palliation is associated with numerous complications during long-term. The Fontan conversion operation has been advocated as an option to avoid some of these problems by converting classical Fontan types to modern forms of the circulation. Early mortality of Fontan conversion, however, remains unclear as available reports include limited numbers of patients and the results are heterogeneous. METHODS We reviewed all original articles from 1994 to 2016 reporting Fontan conversion operations. Reports were analysed with specific reference to patient demographics, patient number, concomitant arrhythmia surgery, pacemaker implantation and early mortality. RESULTS Overall, 37 Fontan conversion studies with a total of 1182 patients were analysed, including 35 single-centre studies and 2 registers. In the 35 single-centre studies the average age at the time of conversion was 21.6years (range 10.2-30.9years). Concomitant arrhythmia operation was performed in 71.6% of patients and concomitant pacemaker implantation procedure was performed in 59.3% of patients. Early mortality varied greatly between publications ranging from 0 to 21%. Based on a random and a fixed effect model mean mortality was 5.3% and 6.2%, respectively. Lower mortality was observed in series including younger patients at the time of conversion (average age<20years, 4.6%) and in the highest volume centre (1.4%). CONCLUSION Fontan conversion carries a substantial mortality risk. However, results vary between centres. Overall, the combination with arrhythmia surgery seems to be associated with lower early mortality especially when patients are referred at an earlier age and are treated at highly experienced centres.

International collaborative initiative towards improving the lives of patients after the Fontan operation: A call for action

a Adult Congenital Heart Centre and Centre for Pulmonary Hypertension, Royal Brompton Hospital, London, UK b Division of Adult Congenital and Valvular Heart Disease, Department of Cardiovascular Medicine, University Hospital Muenster, Germany c Division of Valvular Heart Disease and Adult Congenital Heart Disease, Department of Cardiovascular Medicine, University Hospital Centre Zagreb, Croatia d National Heart and Lung Institute, Imperial College, London, UK

Pulmonary arterial hypertension in adult congenital heart disease

Pulmonary arterial hypertension (PAH) is commonly associated with congenital heart disease (CHD) and relates to type of the underlying cardiac defects and repair history. Large systemic to pulmonary shunts may develop PAH if untreated or repaired late. PAH, when present, markedly increases morbidity and mortality in patients with CHD. Significant progress has been made for patients with Eisenmenger syndrome in pathophysiology, prognostication and disease-targeting therapy (DTT), which needs to be applied to routine patient care. Patients with PAH–CHD and systemic to pulmonary shunting may benefit from late defect closure if pulmonary vascular resistance (PVR) is still normal or near normal. Patients with PAH and coincidental defects, or previous repair of CHD should be managed as those with idiopathic PAH. Patients with a Fontan circulation, despite not strictly fulfilling criteria for PAH, may have elevated PVR; recent evidence suggests that they may also benefit from DTT, but more data are required before general recommendations can be made. CHD–PAH is a lifelong, progressive disease; patients should receive tertiary care and benefit from a proactive DTT approach. Novel biomarkers and genetic advances may identify patients with CHD who should be referred for late defect closure and/or patients at high risk of developing PAH despite early closure in childhood. Ongoing vigilance for PAH and further controlled studies are clearly warranted in CHD.

Post-capillary Pulmonary Hypertension in ACHD

Pulmonary hypertension (PH) is a haemodynamic and pathophysiological condition defined as an increase in mean pulmonary arterial pressure at rest ⩾ 25 mmHg as assessed by right heart catheterization. Haemodynamically PH can be further distinguished into pre-capillary PH depending on the presence of a raised left atrial or pulmonary wedge pressure. Post-capillary PH is common in patients with congenital heart disease, in whom systemic ventricular dysfunction, valve disease, outflow tract obstruction or pulmonary venous stenosis can lead to a rise in post-capillary pressures. In this chapter, we discuss the pathogenesis, impact and management of postcapillary PH in congenital heart disease.

Impact of short-term high altitude exposure on exercise capacity and symptoms in Fontan patients

The Fontan operation was introduced in 1971 and represented a milestone in the management of congenital heart disease with univentricular heart physiology. Even today it remains the main palliation and a life-saving procedure for children born with a functionally univentricular cardiac anatomy that is not amenable to biventricular repair. The surgical palliation consists of the separation of the systemic and pulmonary venous returns without the use of a subpulmonary ventricle. Since its original description with an atriopulmonary anastomosis, multiple modifications of the procedure have been applied.1 Today, more than 40 years after the initial procedure, we are witnessing a substantial number of adult patients living with Fontan circulation. All Fontan variants share similar physiology in which one effective ventricle delivers oxygenated blood to the systemic circulation and gradient between central venous pressure and left atrial pressure drives passively deoxygenated blood into the pulmonary arteries and the lungs. The Fontan physiology is —in part—paradoxical in that it imposes systemic venous hypertension with concomitant pulmonary …

Double inlet left ventricle with unrestricted pulmonary blood flow and survival into adulthood

A 21-year-old male refugee from Syria with known but ill-defined congenital heart disease, presented with palpitations, no signs of heart failure, and near normal oxygen saturation. Chest radiography showed a globally enlarged heart with prominent hilar and pulmonary vessels ( Panel A ). Transthoracic echocardiography revealed a double-inlet-left-ventricle (DILV) with good systolic function ( Panel B ), malposition of the …