Univentricular hearts not undergoing Fontan: the type of palliation matters.

INTRODUCTION AND OBJECTIVES: There is scarce information on patients with single ventricle physiology (SVP) and restricted pulmonary flow not undergoing Fontan circulation. This study aimed to compare survival and cardiovascular events in these patients according to the type of palliation. METHODS: SVP patient data were obtained from the databases of the adult congenital heart disease units of 7 centers. Patients completing Fontan circulation or developing Eisenmenger syndrome were excluded. Three groups were created according to the source of pulmonary flow: G1 (restrictive pulmonary forward flow), G2 (cavopulmonary shunt), and G3 (aortopulmonary shunts±cavopulmonary shunt). The primary endpoint was death. RESULTS: We identified 120 patients. Mean age at the first visit was 32.2 years. Mean follow-up was 7.1 years. Fifty-five patients (45.8%) were assigned to G1, 30 (25%) to G2, and 35 (29.2%) to G3. Patients in G3 had worse renal function, functional class, and ejection fraction at the first visit and a more marked ejection fraction decline during follow-up, especially when compared with G1. Twenty-four patients (20%) died, 38 (31.7%) were admitted for heart failure, and 21 (17.5%) had atrial flutter/fibrillation during follow-up. These events were more frequent in G3 and significant differences were found compared with G1 in terms of death (HR, 2.9; 95%CI, 1.14-7.37; P=.026) and atrial flutter/fibrillation (HR, 2.9; 95%CI, 1.11-7.68; P=.037). CONCLUSIONS: The type of palliation in patients with SVP and restricted pulmonary flow not undergoing Fontan palliation identifies distinct profiles. Patients palliated with aortopulmonary shunts have an overall worse prognosis with higher morbidity and mortality.

Atypical presentation of scimitar syndrome with severe hepatomegaly: a case report.

BACKGROUND: Scimitar syndrome is a rare congenital disease characterized by partial or total anomalous pulmonary venous return from the right lung into the systemic venous system, and accounts for 0.5-2% of all congenital heart disease. Severe forms of the disease are diagnosed in childhood. However, because of the benign form of the syndrome in adults, many are asymptomatic, or present only mild symptoms including exertional dyspnoea, arrhythmias, and respiratory infections. We report an atypical presentation with hepatomegaly. CASE SUMMARY: A 24-year-old woman was evaluated for abdominal discomfort. Physical examination revealed a remarkable hepatomegaly. Chest X-ray revealed dextroversion, enlargement of the right cavities, and a curvilinear opacity known as 'scimitar sign'. A transthoracic echocardiography demonstrated right ventricular dilation and a venous collector draining into right suprahepatic vein, which was severely dilated, with large hepatomegaly. Scimitar syndrome was confirmed by magnetic resonance imaging (MRI). Therefore, the patient underwent surgery, redirecting the pulmonary venous return to left atrium. Three months later, the patient remained asymptomatic and both the hepatomegaly and the right chamber volumes normalized. DISCUSSION: Abdominal discomfort, as in our clinical case, is a highly atypical presentation of scimitar syndrome. It is important for physicians to be aware that diagnostic suspicion can be established from a chest X-ray, on which the scimitar sign can be distinguished in many cases. The diagnosis must be confirmed with other imaging modalities, such as echocardiography, MRI, or computed tomography. Corrective surgery may relieve the symptoms related to liver congestion at follow-up.

Case report of asymptomatic very late presentation of ALCAPA syndrome: review of the literature since pathophysiology until treatment.

BACKGROUND: Anomalous origin of the left coronary artery (LCA) from the pulmonary artery (ALCAPA) is an unusual congenital heart defect which affects approximately 1 in 300 000 live births and accounts for 0.5% of all congenital heart disease. Without surgical intervention, most patients die in infancy (nearly 90%). CASE SUMMARY: We present a rare case of an asymptomatic 67-year-old female. Transthoracic echocardiography demonstrated a dilated right coronary artery (RCA) and multiple collaterals. ALCAPA was confirmed by multidetector computed tomography. The left main artery was seen originating from the pulmonary artery and well-developed collaterals were visualized between the RCA and LCA. No areas of myocardial infarction were identified on cardiac magnetic resonance. Stress studies showed no inducible ischaemia. DISCUSSION: Our clinical case of an ALCAPA patient who survived and remained asymptomatic to their late 60's, highlights the importance of well-collateralized and pressurized coronary system to maintain adequate myocardial perfusion. Physicians should be aware of this congenital anomaly as appropriate early diagnosis is crucial to prevent irreversible myocardial damage, acute ischaemia, and arrhythmias, and can improve patient outcomes. Surgical treatment is suggested irrespective of symptomatology or the presence of inducible myocardial ischaemia.

Predictors of atrial tachyarrhythmias in adults with congenital heart disease.

BACKGROUND: Atrial tachyarrhythmias (ATs) represent the major late complications of congenital heart diseases (CHDs) following surgery. Little is known about the association between echocardiographic parameters and AT. AIMS: This study aimed to investigate a potential correlation among clinical, echocardiographic, and electrocardiographic parameters and AT as well as to analyze outcomes in adults with CHD and AT. METHODS: A retrospective case­control study was performed in adults with CHD. We included 71 patients with AT and 71 control individuals matched by sex, age, and the type of CHD without AT, all from the same institute. Medical records, electrocardiograms, and echocardiograms were reviewed. Adverse cardiovascular events were recorded and defined as cardiovascular mortality, admission for heart failure, or stroke. The univariate and multivariate logistic regression analysis of possible risk factors and the Kaplan-Meier analysis of adverse cardiovascular events were performed. RESULTS: Subpulmonary ventricular systolic pressure≥40 mm Hg (hazard ratio [HR], 6.8; 95% CI, 2.4-18; P <0.001), right atrial dilatation≥21 cm2 (HR, 3.1; 95% CI, 1.2-7.6; P = 0.01), and significant tricuspid regurgitation (HR, 4; 95% CI, 1.3-10; P = 0.02) were identified as the main risk factors for AT. Patients with AT had worse outcomes, more frequently developed adverse cardiovascular events (86% vs 14%; P <0.01), and exhibited a 58% event­free survival rate compared with 98% of the patients without AT after 8 years of follow­up (log rank, 6.6; P = 0.01). CONCLUSIONS: Among patients with CHD, the main risk factors for AT include right atrial dilatation, high subpulmonary ventricular systolic pressure, and significant tricuspid regurgitation. The presence of AT may increase the risk of adverse cardiac events.