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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.

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El bosentán, antagonista oral de los receptores de la endotelina, ha demostrado beneficio clínico en los pacientes en situación de Eisenmenger en el estudio BREATHE-5, lo que ha permitido la aprobación de su uso para tal indicación por la EMEA (Agencia Europea de Medicamentos). Sin embargo, en este estudio el seguimiento se limitó a 16 semanas y excluyó a los pacientes con cardiopatías congénitas complejas (CCC). Valoramos el efecto a largo plazo del tratamiento con bosentán en 10 pacientes con CCC en situación de Eisenmenger. Con un seguimiento clínico medio de 25 meses, todos los pacientes alcanzaron las dosis óptimas sin efectos colaterales ni variación en la saturación arterial de oxígeno en reposo o al máximo esfuerzo. Sí hubo cambios significativos en los parámetros clínicos: la clase funcional NYHA mejoró de 3,3 ± 0,7 a 2,5 ± 0,9 (p = 0,002) y la distancia recorrida en el test de la marcha de los 6 minutos (TM6M) de 266 ± 161 m a 347 ± 133 m (p = 0,015) (AU)

The BREATHE-5 study demonstrated that bosentan, an oral endothelin receptor antagonist, provides clinical benefits in patients with Eisenmenger’s syndrome. As a result, the European Medicines Agency (EMEA) approved its use for this indication. However, follow-up in that study was limited to 16 weeks and patients with complex congenital heart disease were excluded. We assessed the effect of long-term bosentan treatment in 10 patients with complex congenital heart disease and Eisenmenger’s syndrome. In the mean clinical follow-up period of 25 months, all patients reached the target dose without developing side effects and without experiencing a change in arterial oxygen consumption at either rest or maximal exercise. Moreover, there were significant changes in clinical parameters: NYHA functional class improved from 3.3 (0.7) to 2.5 (0.9) (P=.002) and the 6-minute walk distance increased from 266 (161) m to 347 (133) m (P=.015) (AU)

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The BREATHE-5 study demonstrated that bosentan, an oral endothelin receptor antagonist, provides clinical benefits in patients with Eisenmenger's syndrome. As a result, the European Medicines Agency (EMEA) approved its use for this indication. However, follow-up in that study was limited to 16 weeks and patients with complex congenital heart disease were excluded. We assessed the effect of long-term bosentan treatment in 10 patients with complex congenital heart disease and Eisenmenger's syndrome. In the mean clinical follow-up period of 25 months, all patients reached the target dose without developing side effects and without experiencing a change in arterial oxygen consumption at either rest or maximal exercise. Moreover, there were significant changes in clinical parameters: NYHA functional class improved from 3.3+/-0.7 to 2.5+/-0.9 (P=.002) and the 6-minute walk distance increased from 266+/-161 m to 347+/-133 m (P=.015).

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A 39 year-old woman diagnosed with anomalous drainage of middle and lower right pulmonary veins to the inferior vena cava was corrected surgically by means of baffle with patch up to the left atrium. Early after the operation the patient related intolerance to small efforts and an episode of syncope. The cardiac catheterization demonstrated the presence of a severe stenosis in the inferior vena cava, in its union with the right atrium, that was successfully treated by means of intraluminal percutaneous dilation with a catheter of Inoue. After the procedure the gradient decreased and she improved tolerance to effort, which persisted 10 months later.

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INTRODUCTION AND OBJECTIVES: Ruptured sinus of Valsalva aneurysm to right cardiac chambers is an uncommon lesion in Western countries. The prognosis is usually serious unless the condition is promptly treated surgically. For this reason an accurate anatomical and functional evaluation is necessary. The main purpose of this report is to compare the usefulness of multiplane transesophageal echocardiography with transthoracic echocardiography and angiocardiography in the preoperative evaluation of ruptured sinus of Valsalva aneurysm to right chambers. PATIENTS AND METHOD: Since January 1990, 9 patients (mean age 36,3 18 yr, 6 males) with ruptured sinus of Valsalva aneurysm to right chambers were studied. The pathogenesis was congenital aneurysm in 6 patients, aortic prosthesis endocarditis in one and two cases of iatrogenia: during a percutaneous mitral valvuloplasty and after cardiac surgery. Transthoracic echocardiography was performed in all cases, transesophageal echocardiography in 7 and angiocardiography in 8. Two patients died before surgery, and 7 were successfully operated on. RESULTS: Transesophageal echocardiography was more useful when compared to transthoracic echocardiography and angiocardiography in detecting: a) the fistula; b) the sinus involved; c) the right chamber affected; d) congenital aneurysms morphology and size; e) aneurysm prolapse through a ventricular septal defect, y f) the identification of other cardiac congenital or acquired anomalies. CONCLUSIONS: Multiplane TEE is the most accurate tool in the preoperative evaluation of ruptured sinus of Valsalva aneurysm to right chambers.

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