Current Treatment Options for the Failing Fontan Circulation.

The Fontan operation was introduced in 1968. For congenital malformations, where biventricular repair is unsuitable, the Fontan procedure has provided a long-term palliation strategy with improved outcomes compared to the initially developed procedures. Despite these improvements, several complications merely due to a failing Fontan circulation, including myocardial dysfunction, arrhythmias, increased pulmonary vascular resistance, protein-losing enteropathy, hepatic dysfunction, plastic bronchitis, and thrombo-embolism, may occur, thereby limiting the life-expectancy in this patient cohort. This review provides an overview of the most common complications of Fontan circulation and the currently available treatment options.

Echocardiography and MRI parameters associated with exercise capacity in patients after the arterial switch operation.

BACKGROUND: The arterial switch operation (ASO) for transposition of the great arteries has excellent survival, but a substantial number of patients suffer from a reduced exercise capacity. The goal of this study was to identify imaging parameters associated with a reduced exercise capacity in patients after ASO. METHODS: A retrospective analysis was performed of ASO patients who underwent cardiopulmonary exercise testing (CPET) between 2007 and 2017. Reduced exercise performance was defined as a reduced workload peak (Wpeak) with Z-score <-2 or a peak oxygen uptake indexed for weight (VO2peak/kg) with Z-score <-2. Data on echocardiography and cardiac magnetic resonance performed within 1 year of the CPET were collected for comparison. RESULTS: A total of 81 ASO patients (age 17±7 years) were included. Reduced exercise performance was found in 22 patients (27%) as expressed by either a reduced Wpeak and/or a reduced VO2peak/kg. Main pulmonary artery gradient and tricuspid regurgitation gradient by echocardiography were found to be associated with reduced Wpeak (p=0.031; p=0.020, respectively). The main pulmonary artery gradient and tricuspid regurgitation gradient by echocardiography were found to be associated with reduced VO2peak/kg (p=0.009; p=0.019, respectively). No left ventricular parameters were found to be associated with abnormal exercise performance. CONCLUSION: This study demonstrates that ASO patients frequently experience reduced exercise capacity. Echocardiographic evidence of main pulmonary artery stenosis and increased right ventricular pressure were associated with reduced exercise capacity, and are therefore key to monitor during serial follow-up of ASO patients.

Percutaneous Pulmonary Valve Implantation: Current Status and Future Perspectives.

Patients with congenital heart disease (CHD) with right ventricle outflow tract (RVOT) dysfunction need sequential pulmonary valve replacements throughout their life in the majority of cases. Since their introduction in 2000, the number of percutaneous pulmonary valve implantations (PPVI) has grown and reached over 10,000 procedures worldwide. Overall, PPVI has been proven safe and effective, but some anatomical variations can limit procedural success. This review discusses the current status and future perspectives of the procedure.

Anomalous coronary artery originating from the opposite sinus of Valsalva (ACAOS), fractional flow reserve- and intravascular ultrasound-guided management in adult patients.

OBJECTIVES: To describe the use of fractional flow reserve (FFR) and intravascular ultrasound (IVUS) in the evaluation of patients with anomalous coronary arteries originating from the opposite sinus of Valsalva (ACAOS). BACKGROUND: ACAOS of the right and left coronary are rare, but may lead to symptoms and impose a risk for sudden cardiac death, depending on several anatomical features. Assessment and risk estimation is challenging in (nonathlete) adults, especially if they present without symptoms or with atypical complaints. METHODS: The team retrospectively studied 30 consecutive patients with ACAOS with interarterial course, who received IVUS- and FFR-guided treatment at our institution between October 2010 and September 2017. RESULTS: FFR was abnormal in only seven patients. IVUS showed the typical slit-like anatomy of the orifice in 23 patients. Based on FFR and/or IVUS results, in conjunction with the clinical presentation, clinical decision was made. A decision for intervention was made if at least two out of three entities were abnormal. Intervention implied unroofing of the coronary artery (n = 10) or coronary artery bypass grafting (n = 1). In all other patients a conservative strategy was followed. No adverse events occurred in the total population after a median of 37 (0-62) months of follow-up. CONCLUSIONS: Conservative treatment may be justifiable in adult patients with ACAOS in the presence of normal FFR and nonsuspicious symptoms, despite the presence of an interarterial course and/or slitlike orifice on IVUS. We recommend the use of FFR and IVUS in the standard work-up for adult patients with ACAOS and propose the use of a flowchart to aid in decision-making.