[One-year outcome of Senning operation in children with transposition of the great vessels]

Nowadays, the treatment of choice for anatomical correction of transposition of great arteries is arterial switch but some children are not good candidates for this operation. In these cases atrial switch or Senning procedure is an accepted method, thus outcome of this procedure needs to be better delineated. This prospective study included 65 patients that underwent Senning operation in Shahid Rajaee Hospital in Tehran, Iran from 2002 to 2009 and were followed-up for one year. The early and late mortality rates were 16.9% and 1.5%, respectively. 51.8% of early deaths were due to simple Senning procedure, 38.9% due to a Senning with the closure of ventricular septal defect, with or without the repair of pulmonary artery stenosis, 9.3% related to palliative Senning and one late death due to simple Senning. The most common arrhythmia was accelerated junctional rhythm [18.5%]. 15% of cases had Senning pathway obstruction and 1.7% had baffle leaks. Regardless of the mortalities, three patients [5.7%] had significant tricuspid regurgitation. Right ventricular systolic dysfunction was found in 13.3% of the cases, primarily in Senning with ventricular septal defect closure [37.5%]. One case [1.9%] had visible cyanosis and three [5.7%] had exertional dyspnea but 94.3% of cases remained in functional class I. Since the majority of deaths following Senning procedure occur during the first month, especially on the second day post-operatively, assiduity during early post-operative period is crucial. Tricuspid regurgitation or right ventricular systolic dysfunction was not detected in most patients, suggesting optimistic prognosis for these patients

Growth status of Iranian children with hemodynamically important congenital heart disease

The relationship between congenital heart disease [CHD] and growth retardation is well documented. We investigated the growth condition of Iranian children with several types of congenital heart disease [CHD] and compared it with worldwide researches. Growth condition was investigated in 469 patients with important CHD aged from 1 month to 18 years. The patients were divided into two groups; infants [aged 12 months or less], and children [1-18 yrs of age]. Children with hemodynamically unimportant small VSDs or small ASDs were not studied. Other exclusion criteria were prematurity, known genetic disorders and neurologic disease affecting growth. All patients' cardiac diagnoses were made on the basis of clinical and laboratory examinations; including electrocardiography, echocardiography, cardiac catheterization, and angiography. Body weight and height of all patients were measured using conventional methods and compared with standard growth charts. In all patients body weights and heights were significantly lower than normal population. This difference was greater in the weight of female children. Other risk factors for growth failure were large left-to-right intracardiac shunts, pulmonary hypertension and cyanosis. Iranian children with CHD have growth failure somewhat different from other countries. Lower body weights of cyanotic patients and female children indicated that these patients need more nutritional and psychosocial attention

Validity of sildenafil test in patients with pulmonary arterial hypertension associated with congenital heart disease according to clinical and ecardiographic parameters

Pulmonary arterial hypertension is a complication of most congenital heart diseases. We sought to assess the effect of sildenafil on patients suffering from pulmonary arterial hypertension in association with congenital heart disease on the basis of clinical and echocardiographic parameters and compare the catheterization and treatment results so as to evaluate the predictive value of sildenafil on the operability of patients.After primary echocardiography, 21 patients were selected for the final study with a diagnosis of moderate-to-large ventricular septal defect and pulmonary artery hypertension. They were divided into 3 age groups: younger than one year, 1 to 2 years, and older than 2 years. Before and one hour after the consumption of sildenafil, the patients had their oxygen saturation and blood pressure measured. Additionally, the patients underwent echocardiography and cardiac catheterization. The patients' operability was determined on the basis of their clinical condition and their response to oxygen inhalation in the catheterization room. Finally, the results of the drug response and final treatment were analyzed statistically. The 21 patients, who had ventricular septal defect and pulmonary arterial hypertension, were comprised of 8 [38%] boys and 13 [62%] girls. The patients aged from 2.5 to 204 months [mean 30 months]. It was clear that the younger patients had a more positive response to the drug. All the patients who had a positive response to the drug were considered operable after catheterization and all of them had a positive response to treatment. There was no significant correlation between operable/inoperable conditions and response to treatment [P value=0.262], while there was a very significant correlation between response to treatment and response to drug [P value=0.005]. According to the results of this study and given the low cost and availability of sildenafil and its oral consumability on the one hand and the availability and non-invasiveness of echocardiography on the other, it seems that sildenafil is useful in determining the pulmonary vascular bed reactivity via echocardiographic parameters and facilitating the decision-making process for surgery in patients with pulmonary arterial hypertension and congenital heart diseases

Transcatheter closure of fenestration after modified fontan operation in children

Fenestration in the modified Fontan operation allows right-to-left shunting, which reduces the Fontan pathway pressure and improves cardiac output. However, on account of the fact that persistent right-to-left shunting results in cyanosis and paradoxical emboli, fenestration closure is recommended after recovery from the Fontan operation. This study recruited 3 patients who underwent the transcatheter closure of the Fontan fenestration with the ASD-Amplatzer because of severe cyanosis and significant intracardiac shunts. Fenestration closure was performed at a mean age of 8 yr [6-12 yr] and average of 15 months after the Fontan operation. Aortic O[2] saturation increased by an average of 17.6% [9-26%]. During more than a two-year period of follow-up [mean: 27 months], two patients had complete occlusion on echocardiography and the other one had a small residual shunt. One of these patients had atrial flutter during the follow-up. The transcatheter closure of the Fontan fenestration is a safe and feasible technique that is effective in elevating systemic O[2] saturation and well-being and confers acceptable growth and development in children

Congenital left ventricular diverticulum associated with ASD, VSD, and epigastric hernia

Congenital left ventricular diverticulum is a rare cardiac malformation. Two categories of congenital ventricular diverticulum have been identified with regard to their localization: apical and non-apical. Apical diverticula are always associated with midline thoraco-abdominal defects and other heart malformations. Non-apical diverticula are always isolated defects. Diagnosis is established by imaging studies such as echocardiography, magnetic resonance imaging, or left ventricular angiography. Mode of treatment has to be individually tailored and depends on clinical presentation, accompanying abnormalities, and possible complications. We report a 10-month-old girl with left ventricular apical diverticulum, large atrial septal defect, two small muscular ventricular septal defects, and pulmonary hypertension, associated with epigastric hernia. This patient underwent total surgical repair for intra-cardiac defects as well as diverticular resection

Supravalvar aortic stenosis in children report of thirteen cases

During 15 years from 1975 to 1990, thirteen cases of supravalvar aortic stenosis were admitted at the pediatrics department of Shahid Rajai Heart Hospital, Tehran. All patients were subjected to cardiac catheterization and an angiocardiography. Patients ranged in age from 3.5 years to 14 years with a mean of 8.7 years. Seventy seven percent of children were male. Eight cases [61.5%] had Williams' syndrome. Hourglass type of supravalvar aortic stenosis was the most common variant [70%], followed by hypoplastic type [15%] and membranous type [5%]. Peripheral pulmonary stenosis was the most common associated anomaly and was observed in 23% of patients. The main purpose of this investigation is to report the results of the study on supravalvar aortic stenosis and its association with Williams' syndrome in a group of Iranian children