Eisenmenger syndrome

Chen H, Xu Z, Wang S, Shen J, et al. Eisenmenger ventricular septal defect: classification, morphology, and indications for surgery. Pediatr Cardiol. 2011 Jan;32(1):17-23.

“This study aimed to examine the definition and indications for surgery, to elucidate the morphologic substrate of aortic regurgitation, and to extrapolate the pathologic mechanisms of subpulmonary stenosis in Eisenmenger ventricular septal defect (EVSD). The study enrolled 160 patients. Preoperative respiratory symptoms and poor growth were present in 41 patients (26%), and 21 patients (13%) required mechanical ventilation. Perimembranous ventricular septal defect (pVSD) had been diagnosed previously for 136 of the patients (85%) at other institutions. Of the 160 patients, 51 (32%) had muscular posteroinferior rims. Aortic regurgitation was experienced by 36 patients (23%), found to be mild in 31 cases (19%) and moderate in 5 cases (3%). None of the patients had severe regurgitation. No aortic valvuloplasty was performed. The significant risk factors for aortic regurgitation were subpulmonary stenosis (p = 0.001) and a muscular posteroinferior rim (p = 0.000). Subpulmonary stenosis was seen in 57 patients (35%), found to be mild to moderate in 42 cases (26%) and severe in 15 cases (9%). Adequacy of the stenosis band was repaired through the tricuspid valve for 57 of these patients. The definition of EVSD should identify it as a subgroup different from pVSD, and it should be closed as soon as it is identified in developing countries. Aortic regurgitation occurs rarely, and aortic valvoplasty should be performed if it exceeds a moderate level. The subpulmonary stenosis can be repaired through the tricuspid valve.”

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Kumar RK, Sandoval J. Advanced pulmonary vascular disease: the Eisenmenger syndrome. Cardiol Young. 2009 Dec;19(6):622-6.

“The Eisenmenger syndrome should be viewed as a multisystem disease. [6] Multiple systems of organs are affected as a result of chronic hypoxia, erythrocytosis, and cardiac failure (Table 1). Infectious complications, such as endocarditis and brain abscess, are common. Diagnostic evaluation should therefore be comprehensive. The commonest symptom is exercise intolerance. Other common symptoms include dyspnoea, syncope, chest pain, cyanosis, and haemoptysis. Pulmonary hypertension is readily evident on physical examination in the form of a loud pulmonary component of the second heart sound, prominent pulmonary arterial pulsations, a right ventricular heave, and in many instances, an early diastolic murmur of pulmonary regurgitation. Additional murmurs of tricuspid regurgitation may be present, but the murmur across the primary cardiac defect disappears. Central cyanosis is usually readily identifiable, and is often accompanied by clubbing. It may be limited to the legs in patient with reversal of the shunt through a patent arterial duct, or only apparent through measurement of saturations of oxygen. This can happen in presence of significant anaemia, or if the nail beds are pigmented.”

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Beghetti M, Tissot C. Pulmonary arterial hypertension in congenital heart diseases. Semin Respir Crit Care Med. 2009 Aug;30(4):421-8.

“Pulmonary hypertension complicates the course of many children and adults with congenital heart diseases (CHDs). The increase in pulmonary pressure associated with CHD is secondary to either increased pulmonary blood flow or increased postcapillary pressures. Pulmonary arterial hypertension is in the vast majority associated with congenital cardiac shunts. Despite major advances in the understanding of the regulation of the pulmonary vascular bed and the pulmonary endothelial lesions leading to pulmonary vascular disease, despite the advances in surgical repair and the discovery of potential therapies in the pre- and postoperative period, pulmonary hypertension still carries a significant mortality and morbidity in patients with CHD. The recent introduction of targeted therapies in other forms of pulmonary arterial hypertension has led to a renewed interest in pulmonary hypertension associated with CHD and this particularly for the most advanced form, the so-called Eisenmenger syndrome (ES). This review summarizes the current knowledge on pulmonary hypertension associated with CHD, focusing on the pathophysiology and treatment of ES.”

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