Right Ventricular Outflow Tract Obstruction by an Aneurysm of the Ventricular Membranous Septum: A Systematic Review of Case Reports.

We performed a literature search to identify the details of patients with right ventricular outflow tract obstruction caused by an aneurysm of the ventricular membranous septum in a perimembranous ventricular septal defect. Thirty-one cases with a median age of 29 years (range, 1-69 years) were studied. A right ventricle-pulmonary artery systolic pressure gradient ranged from 35 to 107 mm Hg (mean 69 mm Hg). An interventricular shunt was absent in eight patients: two children and six adults. It is necessary to monitor the size of an aneurysm of the ventricular membranous septum, whether or not an interventricular shunt is present.

Quadricuspid Pulmonary Valve: Case Report and the Comparison with Quadricuspid Aortic Valve.

BACKGROUND: Quadricuspid pulmonary valve (QPV) is a rare congenital anomaly. Simple QPV had been mainly diagnosed at the time of autopsy before 2000, and the frequency rates of QPV are approximately 0.02%-0.41%. QPV was initially diagnosed using transthoracic echocardiography (TTE) after 2000 and with contrast computed tomography (CT) or cardiac magnetic resonance imaging (CMR) after 2009. Obtaining the cross-sectional view of the pulmonary valve using TTE is difficult. We aimed to review the papers regarding the incidence, embryology, diagnosis, associated congenital heart anomalies, and prognosis in patients with QPV, and furthermore to compare with those in patients with quadricuspid aortic valve (QAV). CASE PRESENTATION: We diagnosed QPV with mild stenosis in a 12-month-old infant. With a slight angulation of the transducer superiorly from the left high parasternal short-axis view, a short-axis view of QPV was obtained. RESULTS: In QPV cases diagnosed at autopsy, Hurwitz's type-b with three equal cusps and one smaller cusp is dominant, whereas Hurwitz's type-a with four equal cusps is dominant in clinically diagnosed cases. Congenital heart anomaly and valvular stenosis are more frequent in patients with QPV than in patients with QAV. Coronary artery anomalies and infectious endocarditis are more frequent in patients with QAV than in patients with QPV. The incidence of PR is more common in type-a QPV than in type-b QPV. There is no difference between type-a QAV and type-b QAV with respect to the incidence of aortic regurgitation (AR). It is assumed that QPV is a risk factor for a Ross operation. However, QPVs have been used as autografts in certain patients. CONCLUSION: Between QPV and QAV, various differences were found in frequency rates, diagnostic methods, valve morphology, valve function, associated congenital heart diseases, and frequencies of infectious endocarditis.

A review of isolated muscular ventricular septal defect.

BACKGROUND: Color Doppler echocardiography greatly facilitates the diagnosis of isolated muscular ventricular septal defect with a small shunt. DATA SOURCES: Original research articles were collected from database, including PubMed and Google scholar. Relevant articles about muscular ventricular septal defect were included. RESULTS: The frequency of isolated muscular ventricular septal defect is 5.7% in preterm infants and 1.1-5.3% in term infants. Spontaneous closure in muscular ventricular septal defect occurs with higher frequency and earlier than in perimembranous ventricular septal defect. Approximately 80-90% of isolated muscular ventricular septal defect closes spontaneously by 12 months of age. Midventricular muscular ventricular septal defect is spontaneously closed earlier in the short term, but no site difference is found in the long term. The spontaneous closure mechanism is regarded as aposition of the muscle tissue or fibrous tissue formation in the right ventricular side, but in rare cases involves aneurysm formation of the fibrous tissue. Regarding spontaneous closure of isolated muscular ventricular septal defect diagnosed for the fetus, further studies are needed. Chromosomal microarray analysis of fetuses with isolated muscular ventricular septal defect has revealed that it is not a severe risk factor of chromosomal abnormalities. CONCLUSIONS: This paper presents a review of the history of the diagnosis and frequency of ventricular septal defect, with discussion of its natural history from the fetal period to after birth in patients with isolated muscular ventricular septal defect.

A case of coronary-pulmonary artery fistula with coronary artery aneurysm detected for Kawasaki disease remote phase.

Follow-up echocardiography showed two coronary-pulmonary artery fistulae and a coronary artery aneurysm in a 12-year-old boy who had been diagnosed with Kawasaki disease without persistent coronary artery lesion at the acute phase when he was 6-months-old. Left coronary arteriogram confirmed the fistulae and the 4.3 × 6.3 mm aneurysm. Results show that the fistula is associated with Kawasaki disease.

Atriofascicular Mahaim with Ebstein anomaly: A case report.

We report a case of a 7-year-old girl with atriofascicular Mahaim (AFM) pathway concomitant with Ebstein's anomaly. The QRS wave showed left bundle branch block pattern on electrocardiogram. Holter electrocardiogram showed prolongation of the PR interval and QRS morphological change during sinus tachycardia. An electrophysiological study demonstrated that the distal His potential appeared earlier than the proximal His potential, which suggested retrograde His conduction toward the atrioventricular node. Conduction from the Mahaim fiber to the His bundle was faster than that from the atrioventricular node towards the His bundle. The findings of this important case allowed a differential diagnosis between AFM and Wolff-Parkinson-White (WPW) syndrome.

Spontaneous closure of muscular trabecular ventricular septal defect: comparison of defect positions.

AIM: To evaluate the timing and frequency of spontaneous closure of the muscular trabecular ventricular septal defect (VSD). METHODS: We performed a historical cohort study for which 150 patients <3 months of age (median age, 9 days) diagnosed as having a muscular trabecular VSD were selected. Median age at latest follow-up was 2.8 years. Another 32 patients diagnosed after 3 months of age were also reviewed. Using colour Doppler, defects were classified into three groups: anterior, apical and midventricular. RESULTS: Spontaneous closure occurred in 126 patients (84%): anterior, 36 of 47 (83%); apical, 26 of 31 (84%); and midventricular, 64 of 72 (89%). Multivariate analyses showed a lower frequency of spontaneous closure for patients of age of ≥ 20 days at initial echocardiography [hazard ratio 0.60, 95% confidence interval (CI) 0.39-0.89] and for anterior and apical muscular trabecular VSD (hazard ratio 0.66, 95% CI 0.47-0.95). The prevalence of the midventricular muscular trabecular VSD was significantly lower in patients ≥ 3 months of age at initial echocardiography than in those < 3 months (p = 0.010). CONCLUSION: We infer that midventricular muscular trabecular VSD tends to close spontaneously earlier and more frequently than either anterior or apical muscular trabecular VSD.

Spontaneous closure of perimembranous ventricular septal defect after school age.

BACKGROUND: The number of studies of long-term follow up to adolescence is very low on spontaneous closure (SC) of perimembranous ventricular septal defects (P-VSD) in children not undergoing surgical closure because of small left-to-right shunting. METHODS: Seventy patients with a P-VSD with pulmonary-to-systemic flow ratio (Qp/Qs) < 1.7 underwent cardiac catheterization at the age of 2-10 years (mean, 5.1 years). Excluding 22 patients who dropped out by 15 years, 48 were selected. Qp/Qs ranged from 1.00 to 1.68 (mean, 1.17). The average follow-up period of 37 patients excluding the SC patients was 17.8 years. They were classified according to Qp/Qs into three groups: group I, 1.4 or= 1.2 showed no tendency to close spontaneously. The factor most influencing SC of P-VSD after school age seemed to be the shunt ratio.

Spontaneous closure of ventricular septal defects followed up from <3 months of age.

BACKGROUND: This study evaluates the incidence and timing of spontaneous closure (SC) of ventricular septal defect (VSD) using Doppler color flow mapping. METHODS: A total of 225 infants (mean age 30 days) were diagnosed with uncomplicated VSD: 31 (14%) subpulmonary VSD, 159 (70%) perimembranous, and 35 (16%) muscular. The patients were divided into two groups according to the presence or absence of congestive heart failure (CHF). SC was confirmed with color Doppler. RESULTS: Surgical closure was performed in 59 patients (26%). SC occurred in 107 patients (48%); three (10%) of 31 with subpulmonary VSD, 75 (47%) of 159 with perimembranous VSD, and 29 (83%) of 35 with a muscular VSD. Average age at SC was 19 months. In three SC patients with a subpulmonary VSD, there was no aortic valve prolapse and no aortic regurgitation. SC occurred in 96% of SC patients with a perimembranous VSD by the age of 6 years, and in 93% of those with a muscular VSD by the age of 3 years. In patients without CHF, the rate of SC was 72%; 23% in subpulmonary VSD, 74% in perimembranous, and 85% in muscular. SC occurred in only 23% of patients with a perimembranous VSD with CHF. Mean age at the final examination was 6.9 years in 59 patients with a VSD remaining open, and 63% of patients with a perimembranous VSD remaining open had an aneurysm of the ventricular membranous septum. CONCLUSIONS: The SC rate of VSD by mean age of 6.9 years was 48%, but it was 72% in patients without CHF. In patients with CHF, SC was seen only in patients with a perimembranous VSD. The rate of SC was 10% in subpulmonary VSD. The authors contend that SC probably occurred by growth of muscular septum surrounding VSD. Muscular VSD spontaneously closed earlier than perimembranous VSD.

Myocardial ischemia in Kawasaki disease: evaluation with dipyridamole stress technetium 99m tetrofosmin scintigraphy.

BACKGROUND: The coronary artery abnormalities in Kawasaki disease (KD) often cause myocardial ischemia. Previous publications have described the use of thallium 201 myocardial perfusion imaging to determine the extent of ischemia in patients with KD. The technetium perfusion agents offer better resolution and may offer additional information about ventricular function in these patients. This study was performed to evaluate myocardial perfusion in patients with KD through use of technetium 99m tetrofosmin (TF) in conjunction with dipyridamole vasodilator stress. METHODS AND RESULTS: Eighty-six patients with KD aged 11.5 +/- 6.4 years and 20 age-matched control patients without heart disease were studied. Among 86 patients with KD, significant coronary artery stenosis (>or=75%) was observed in 20, coronary aneurysm without stenosis in 37, and no coronary lesions in 29. After administration of high-dose dipyridamole infusion (0.70 mg/kg), 74 to 370 MBq TF was injected and 148 to 740 MBq TF was injected at rest 4 hours later. Single photon emission computed tomography images were obtained more than 30 minutes after TF injection. Regional myocardial hypoperfusion was observed in 18 patients who had coronary artery stenosis of 75% or greater (sensitivity, 90%, and specificity, 100%). Two patients did not demonstrate ischemic changes in coronary artery stenosis in group I; one had good collateral flow. There were no TF injection-related complications. CONCLUSIONS: Tetrofosmin myocardial perfusion imaging can detect regional hypoperfusion in children with KD.

Right-to-Left Shunt Through a Ventricular Septal Defect During Sedated Sleep.

A 10-month-old infant with Down's syndrome developed cyanosis due to a right-to-left shunt through a ventricular septal defect during sedated sleep induced for echocardiography. During cardiac catheterization, arterial oxygen saturation decreased, and arterial carbon dioxide concentration increased. Various echocardiographic parameters during sedated and natural sleep were compared. It was considered that hypoventilation caused by the narrowed upper airway was induced by sedated sleep. We conclude that the influence of sedatives should be considered in the estimation of echocardiographic data if an infant with Down's syndrome has a ventricular septal defect with pulmonary hypertension.