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.

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.

Unusual distal right pulmonary artery origin from right ductus arteriosus with uncommon left-sided aortic arch / Inusual origen de la parte distal de la arteria pulmonar derecha en un ductus arterioso derecho en arco aórtico izquierdo no común

Abstract A bilateral persistent ductus arteriosus with right proximal pulmonary artery agenesis is a very unusual anatomic variant. These malformations are assumed to occur during the transformation of the sixth aortic arch with the pulmonary trunk in the development of the aorta and its branches. A 2-months preterm baby was referred by severe bronchopulmonary dysplasia. Transthoracic echocardiography showed a large pulmonary trunk and left pulmonary artery. No right proximal pulmonary artery was found. Cardiac computed tomography study showed bilateral ductus arteriosus. The right proximal pulmonary artery was originated from an arterial structure coming off the right subclavian artery, a right ductus arteriosus. The aortic arch was left-sided, with a peculiar variant at the exit of the supraaortic vessels. Right subclavian artery emerged as the first supraaortic vessel, followed by a common trunk, giving rise to both carotids and the left subclavian artery as the last vessel. Right lung was hypoplastic with interstitial disease. The calibre of the right proximal pulmonary artery was inadequate to perform a corrective surgery. LDA closure was performed, keeping a prostaglandin infusion to maintain right ductus arteriosus patency until right proximal pulmonary artery size will be big enough to perform a successful surgical reconstruction. Unfortunately, right ductus arteriosus became narrow at its origin and she developed severe pulmonary hypertension and the patient died.

Resumen El ductus arterioso bilateral con agenesia proximal de la arteria pulmonar derecha es una variante anatómica rara producida por alteraciones de la transformación del sexto arco aórtico en las arterias pulmonares, la aorta y sus vasos, durante el desarrollo embrionario. Se presenta el caso de un bebé prematuro de dos meses, que fue derivado a nuestro hospital por displasia broncopulmonar severa. El ecocardiograma transtorácico no identificó el origen de la arteria pulmonar derecha. El estudio por tomografía computarizada cardiaca mostró ductus arterioso bilateral e identificó el origen distal de la arteria pulmonar derecha en una rama de la arteria subclavia derecha, identificada como ductus arterioso derecho. El pulmón derecho era hipoplástico y presentaba alteraciones intersticiales. A su vez, el patrón de los vasos supraaórticos era anómalo. El primer vaso correspondió a la arteria subclavia derecha, seguido por tronco común de ambas carótidas y por último la arteria subclavia izquierda. El calibre de la arteria pulmonar derecha fue inadecuado para realizar una cirugía correctiva, por lo cual se decidió cerrar el ductus arterioso izquierdo y mantener infusión de prostaglandina hasta que la arteria pulmonar derecha tuviera un calibre adecuado para la cirugía. Infortunadamente, el ductus arterioso derecho se cerró en su origen, desarrollándose hipertensión pulmonar severa y la paciente falleció.