Sinus node dysfunction is relatively common in patients with Fontan palliation for single ventricle congenital heart disease, and such patients often are in junctional rhythm or may have pacemaker systems for bradycardia. Because the physiologic determinants of left atrial pressure play a major role in determining pulmonary blood flow and therefore cardiac output in Fontan patients, the loss of atrioventricular (AV) synchrony in junctional rhythm or demand ventricular pacing in these patients might be expected to influence cardiac output. We report two cases of Fontan patients with the absence of AV synchrony that resulted in reversal of flow through the Fontan fenestration during ventricular systole. In both cases, restoration of AV synchrony by atrial pacing resulted in the elimination of retrograde fenestration flow, increased cardiac output, and improved clinical status.
Abiotrophia defectiva, one of several nutritionally variant Streptococcus species, is an uncommon but important cause of endocarditis in children. We describe an unusual case complicated by extensive aortitis with pits in the ascending aorta and the proximal aortic arch.
The primary purpose of this study was to determine the tolerability of intermittent intravenous (IV) sildenafil for the treatment of pulmonary hypertension in pediatric patients. Secondary objectives were to evaluate parameters related to efficacy. METHODS: This was a retrospective chart review from January 2013 to August 2014 of pediatric patients under age 18 years treated with intermittent doses of IV sildenafil for pulmonary hypertension. Patients were excluded if they were over age 18 years or received sildenafil for other indications. Measures collected to assess tolerability include blood pressure and heart rate before and after the administration of IV sildenafil, as well as adverse events. RESULTS: Thirty-seven patients (21 females and 16 males) were identified meeting inclusion criteria, and 21 (56.8%) were on oral sildenafil prior to the initial IV dose. The mean decrease in blood pressure after the first dose of IV sildenafil was 7.16/2.74 mmHg. The decrease in systolic blood pressure was statistically significant. During the study period, 5 patients experienced medication related adverse events, primarily hypotension. Despite this, none of the patients had the medication discontinued due to these events. For secondary objectives, a statistically significant difference was not found between other clinical measures before and after intermittent IV sildenafil dosing. CONCLUSIONS: Sildenafil, when administered as intermittent IV doses, was tolerated by the majority of patients evaluated in this study. For pediatric patients with pulmonary hypertension in whom enteral or continuous IV sildenafil cannot be administered, intermittent IV sildenafil may be considered as an alternative administration option.
A 14-YEAR-OLD WHITE FEMALE WAS BORN AT 37 weeks of gestation with a balanced Rastelli Type A atrioventricular septal defect. There was mild sub-aortic narrowing. There was a persistent left superior caval vein to a dilated coronary sinus. The inferior caval vein was not identified, and the abdominal aorta was to the left of the spine. A large azygous vein was seen draining to the right superior caval vein. The pulmonary venous connections were normal.At 11 weeks of age, she underwent two-patch repair of atrioventricular septal defect with reconstruction of the mitral valve and tricuspid valves. At 13 weeks, repeat mitral valve repair was performed. At 31 months, an additional repeat mitral valvuloplasty was performed. At 34 months, mitral valve replacement with a No. 21 Saint Jude prosthesis was carried out. A dual-chamber epicardial pacemaker was implanted for post-surgical heart block. Culture of the native mitral valve was found to be positive for bacillus infection and she was treated with clindamycin. At 9 years of age, she underwent mitral valve replacement with a No. 25 Saint Jude prosthesis, and septal myectomy through the aortic valve.The patient has been clinically stable and doing well for 5 years. Serial echocardiograms were performed and demonstrated stable cardiac findings. Intact atrial and ventricular patches, normally functioning No. 25 Saint Jude prosthesis in the mitral position, no residual or recurrent sub-aortic obstruction, mild aortic valve regurgitation, interrupted inferior caval vein with azygous continuation to the superior caval vein, mild left ventricular enlargement with slightly abnormal/dyssynchronous systolic contractility.At 13 years of age, a routine complete echocardiogram was performed including three-dimensional imaging. 1 Using an IE33 system (Philips Medical Systems, Andover, Massachusetts, United States of America), two-and three-dimensional imaging was accomplished. The S5-sector array probe was used for the two-dimensional imaging and the X3-1 matrix array probe for the three-dimensional imaging.In this study, an echolucent area lateral to the aortic valve and contiguous with the left ventricular outflow tract could be seen, and measured approximately 17 millimetre by 22 millimetre. This area appeared to expand and contract with ventricular systole and diastole. Clip 1: two-dimensional parasternal short axis.In some video clips, this echolucent area seemed to be in communication with the left anterior descending coronary artery, and was first thought to be a coronary artery aneurysm. This area could not be conclusively seen from other views. Clip 2: twodimensional parasternal short axis sliding medially towards the aortic root.Using the matrix array three-dimensional transducer, several full-volume data sets were obtained from the parasternal window, taking care to use settings that yield the largest area of view and with the highest frame rate. The data sets were obtained with held respirations over four cardiac cycles.
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