Membranous septal aneurysm: an unusual cause for right ventricular outflow tract obstruction in a malaligned ventricular septal defect with aortomitral discontinuity (double-outlet right ventricle) associated with visceral heterotaxy

The development of a septal aneurysm in the natural history of membranous ventricular septal defects usually makes the defect hemodynamically less significant. This report describes a case of severe right ventricular outflow obstruction produced by a membranous septal aneurysm in a patient who had an anterior malaligned ventricular septal defect with aortomitral discontinuity (double-outlet right ventricle). This patient did not have pulmonary stenosis other than the dynamic obstruction produced by the septal aneurysm. In this patient, the septal aneurysm produced both favorable and unfavorable hemodynamic effects. A reduction in the size of the ventricular septal defect produced a favorable effect, whereas a right ventricular outflow obstruction led to the unfavorable situation of right ventricular hypertension and hypertrophy. The large septal aneurysm in the presence of an already compromised right ventricular outflow tract related to an anteriorly malaligned septum resulted in severe obstruction.     

ECHOCARDIOGRAPHIC DIAGNOSIS OF AN ANEURYSM OF THE MEMBRANOUS VENTRICULAR SEPTUM

Abstract. A patient with a small ventricular septal defect complicated by an aneurysm of the membranous ventricular septum is reported. The aneurysm was diagnosed by left ventricular angiocardiography and, non-invasively, by echocardiography. The clinical significance of the anomaly is discussed in relation to the pertinent literature.     

Dissecting ventricular septal aneurysm after open-heart surgery

Pseudoaneurysm of the muscular ventricular septum has been reported after myocardial infarction and after transaortic septal myotomy. In our case, surgical repair for infundibular pulmonary stenosis and membranous ventricular septal defect (VSD) was complicated by a dissecting ventricular septal aneurysm with VSD. Echocardiography was the only diagnostic procedure that could demonstrate the lesion. ECG and body surface mapping suggested a local septal infarction, its etiology most probably due to the closing sutures of the initial VSD.     

A Rare Case of Left Lung Hypoplasia Associated With Congenital Pulmonary Artery Aneurysm and Ventricular Septal Defect

This report describes a 3-year-old boy who presented with hemoptysis and a history of congenital heart disease detected at the age of 3 months. Clinical and laboratory evaluation showed a large true congenital pulmonary artery aneurysm of the left main pulmonary artery occupying the entire left hemithorax and two small aneurysms in the right lung. In addition, a restricted ventricular septal defect was detected. It was evident on clinical and histologic grounds that the multiple aneurysms seen in this child stemmed from congenital weakness of the arterial wall in conjunction with increased pulmonary blood flow and elevated pulmonary artery pressure.     

Pulmonary atresia with obstructed ventricular septal defect

Summary Two patients with pulmonary atresia had a loud holosystolic murmur and an obstructed ventricular septal defect. Case 1 simulated pulmonary atresia with intact ventricular septum, presenting at 3 months of age with venous congestion, severe tricuspid regurgitation, and suprasystemic right ventricular pressure. Case 2 had a right ventricular systolic pressure that was initially at the systemic level but had increased to a suprasystemic level by 3 years of age. Autopsy showed that the ventricular septal defect was large in each case but was obstructed by tricuspid valve tissue in case 1 and by a hypertrophied septal band in case 2.Supported in part by the University of Illinois Foundation Goodenberger Medical Research Grant (2-44-33-66-3-14)     

Effects of interventricular shunt on indices of left ventricular function

The accuracies of indices of left ventricle function were examined in an open-chest model in dogs with and without a ventricular septal defect, in which the ventricular shunt was opened and reclosed by a specially designed flowmeter probe with a cap. The systolic time interval, the maximal rate of pressure development in the left ventricle (+LV dP/dt), +LV dP/dt corrected for the isometric pressure (+LV dP/dt/Pd), and the time to +LV dP/dt (t-dP/dt) were determined by recording the aortic flow, ventricular shunt flow, aortic pressure, pulmonary arterial pressure, and left ventricular pressure. The isometric contraction time, the preejectional period, and the ejection time shortened with decrease of the mean aortic pressure and aortic flow, and the mean pulmonary arterial pressure increased after opening the ventricular shunt. When the pulse was varied by atrial pacing, the systolic time interval was affected in dogs both with and without a ventricular septal defect, but "isometric contraction time" was not affected in animals with a ventricular septal defect. Dopamine and methoxamine were used to evaluate the effects of the inotropic state and afterload on these indices. The extents of the changes in the systolic time interval and +LV dP/dt were different in animals with and without a ventricular septal defect, but the changes in preejectional period/ejection time, +LV dP/dt/Pd and t-dP/dt were similar in the two conditions. These results suggest that the systolic time interval and the indices of left ventricular pressure are useful in assessment of cardiac function only in certain conditions.     

Is early surgery always necessary in patients with aortic valve prolapse complicating an Eisenmenger-type ventricular septal defect?

Abstract Background. The report presents three patients who showed a typically prolapsed aortic cusp with or without aortic regurgitation associated with a malalignment-type peri membranous defect, the so called Eisenmenger-type ventricular septal defect.
Results. Each patient developed spontaneous complete or near closure of the ventricular septal defect without worsening of the aortic regurgitation.
Conclusions: These cases suggest that prolapsed aortic cusp complicating a malalignment perimembranous ventricular septal defect is not always an absolute indication for early surgery to prevent progressive aortic regurgitation.     

Congenital aneurysm of the muscular interventricular septum in a child

Congenital aneurysms of the muscular interventricular septum without ventricularseptal defect are extremely rare. We describe a five-month-old girl patient with congenital aneurysm, pathological ST changes and minimal right ventricular outflow tract obstruction. The electrocardiogram showed Wolf-Parkinson-White syndrome, pathological ST changes and combined ventricular hypertrophy criteria. The transthoracic echocardiography and cardiac catheterization demonstrated a septal aneurysm which was bulging into the right ventricle and systolic pressure gradients between the main pulmonary artery and right ventricle outflow tract with normal coronary arteries. The patient has shown no symptoms, but some rhythm disturbances may be expected because of her electrocardiographic anomalies. Therefore, these patients should be followed carefully because of possible complications.     

Comparison of types of pulmonary stenosis with the state of the ventricular septum in complete transposition of the great arteries

Summary The pulmonary valve and left ventricular outflow tract of 214 hearts with d-transposition of the great arteries (d-TGA) were visually inspected (126 of 214 with intact ventricular septum and 88 of 214 with ventricular septal defect [VSD]). Pulmonary stenosis (PS), either valvular, subvalvular, or in combination, was present in 26 cases and was found to be more common in the presence of a VSD (20.5%) than intact ventricular septum (6.3%). PS occurred more commonly in the presence of a supracristal VSD than an infracristal VSD (70%, 7 of 10 vs 15%, 10 of 66). Further, infracristal or supracristal VSDs were associated with different types of pulmonary obstruction. In seven of ten cases with infracristal VSD and PS, the stenosis was caused by an anomaly of an atrioventricular valve. In six of seven cases with supracristal VSD and PS, the latter was caused by membranous encirclement of the left ventricular outflow tract. * Presented in part as an abstract at the American Heart Association Scientific Sessions, Fall 1987.     

Clinical implication of plasma natriuretic peptides in children with ventricular septal defect

BACKGROUND: There is little information available concerning plasma concentrations of B-type natriuretic peptide (BNP) in children with a ventricular septal defect. The aim of the present study was to determine hemodynamic factors that control plasma concentrations of BNP and the clinical implications of BNP compared with atrial natriuretic peptide (ANP) in children with ventricular septal defect. METHODS: Fifty-nine patients with ventricular septal defect (28 boys and 31 girls) without pulmonary vascular disease were enrolled. The patients' ages ranged from 3 months to 13 years (mean 3.1 years). Plasma BNP and ANP were determined by immunoradiometric assay. Hemodynamic variables derived from cardiac catheterization were analyzed in terms of correlation with BNP and ANP. RESULTS: It was found that plasma BNP significantly positively correlated with ANP (ANP = 2.1 x BNP + 25 pg/mL; r = 0.81, P < 0.0001) and BNP never exceeded ANP in the present patient series. Plasma BNP as well as ANP significantly positively correlated with pulmonary to systemic flow ratio (r = 0.65 and r = 0.59, respectively) and mean pulmonary artery pressure (r = 0.72 and r = 0.68, respectively). In addition, plasma BNP of > or =20 pg/mL and ANP of > or =50 pg/mL identified children with mean pulmonary artery pressure of > or =20 mmHg with a sensitivity of 82% and 97%, respectively, and a specificity of 89% and 84%, respectively. CONCLUSION: Plasma BNP and ANP reflect pressure and volume loads to the pulmonary artery and right ventricle and may help to identify children with ventricular septal defect complicated by pulmonary hypertension that demands early intervention.     

Coarctation of the aorta in the syndrome of absent pulmonary valve with ventricular septal defect

Summary The case of a male child is reported in which the syndrome of absent pulmonary valve and ventricular septal defect was associated with anomalous drainage of the right superior pulmonary vein into the superior vena cava, atrial septal defect, a right aortic arch, and an aortic isthmic coarctation. The combination of aortic coarctation with a high degree of right-ventricular outflow obstruction has not been reported so far in the literature. This case shows that there is no proof of aplasia of the ductus arteriosus in the syndrome of absent pulmonary valve and ventricular septal defect. Most likely, there is an early involution of the ductus arteriosus; persistence of the ductus arteriosus would not be compatible with fetal survival.     

Ventricle works as a converting organ of atrial blood flow: Physiological significance of mean ventricular pressure

The roles and characteristics of the ventricle were examined using mean ventricular pressure (MVP) in ventricular-vascular assocation. One hundred and two patients with congenital heart diseases who had undergone cardiac catheterization were studied. They were divided into five groups: Group 1, atrial septal defect without pulmonary hypertension (PH); Group 2, ventricular septal defect (VSD) without PH; Group 3, VSD with PH; Group 4, pulmonary valvular stenosis; and Group 5 as a control group. Then, we examined the relationships between mean pulmonary artery pressure (MPAP) and mean right ventricular pressure (MRVP), and also between mean systemic arterial pressure (MSAP) and mean left ventricular pressure (MLVP) among the five groups. Furthermore, we defined new indicators to express the ease of blood flow through each ventricle. They were referred to as a conductance of the right ventricle (CD_R) and a conductance of the left ventricle (CD_L), respectively. Then they were compared among the five groups. The values of MPAP/MRVP and MSAP/MLVP were kept constant to be about 1.3 and 1.7, respectively. Furthermore, CD_R was different betweeen each group according to the property of the pulmonary vascular bed, whereas CD_L took almost the same value among the five groups. The ventricle works as a converter of atrial blood flow so that it can achieve efficient blood transport.     

Intrauterine closure of membranous ventricular septal defects: Mechanism of closure in two autopsy specimens

Summary Anatomic evidence of intrauterine closure of ventricular septal defects (VSDs) has been reported rarely. Between 1985 and 1990, 112 autopsies were performed at the Mayo Clinic on third trimester stillborns and infants who died during the first week of life. There were 21 (19%) cases of congenital heart disease. VSD was found in 12 (11%) cases: in eight (7%) as a part of a more complex heart defect and in four (4%) as an isolated lesion. Two cases with membranous VSDs with tricuspid valve tissue partially occluding the ventricular septal defect were found. A 2280-g female infant (case 1) with trisomy 18 died at 4 days of age. Autopsy revealed bilateral superior venae cavae, a large atrial septal defect, cor triatriatum, an atypical tricuspid valve with large septal leaflet partially obstructing a large membranous VSD, a hypoplastic right ventricle, and severe pulmonic stenosis. A 2610-g female infant (case 2), born with congenital heart block died at 4 days of age. Autopsy revealed cor triatriatum dexter obstructing the tricuspid orifice, a large membranous VSD partially obstructed by the septal leaflet of the tricuspid valve, four small muscular VSDs, and pulmonic stenosis. These cases suggest that closure of membranous VSDs may gegin in utero and the mechanism of closure is similar to that reported postnatally.     

Successful Surgical Treatment for a Single Coronary Artery Arising From the Pulmonary Artery

The case of a 17-day-old infant who underwent correction of an aortic coarctation with pulmonary artery banding for ventricular septal defect is reported. Ischemic cardiac failure 3 weeks later led the authors to diagnose a single coronary artery originating from the pulmonary artery. At the age of 2 months, the infant underwent successful reimplantation of the coronary artery onto the ascending aorta and surgical closure of the septal defect. Normalization of the left ventricular function was observed at the 2-year follow-up assessment.     

Surgical Repair for D-Transposition of the Great Arteries Associated With an Aortopulmonary Window Using the Fenestrated Ventricular Septal Defect Patch as a Safety Adjunct

Transposition of the great arteries with a ventricular septal defect and an associated aortopulmonary window is a rare anatomic combination having a high risk for pulmonary hypertension. Arterial switch with closure of the ventricular septal defect and repair of the aortopulmonary window is the procedure of choice, but a postoperative pulmonary hypertensive crisis is a common occurrence associated with significant morbidity and mortality. This report describes one case of such an anatomic lesion, which was repaired successfully with a fenestrated ventricular septal defect patch as an adjunct to decrease the risk of a postoperative pulmonary hypertensive crisis.     

Congenital Aneurysm of the Muscular Interventricular Septum in a Fraternal Case Diagnosed by Fetal Echocardiography

Two siblings with aneurysms of the muscular ventricular septum are presented. Case 1 (the older brother) underwent fetal echocardiography at 26 weeks of gestation. Initially, it was thought that he had a muscular ventricular septal defect; however, postnatal echocardiography revealed an aneurysm of the muscular portion of the ventricular septum. Retrospective review of the 26-weeks gestation fetal echocardiogram confirmed this diagnosis. Case 2 (a younger brother of the first case) was also diagnosed as having an aneurysm of the ventricular septum by fetal echocardiography at 28 weeks of gestation. Postnatal echocardiography confirmed this diagnosis.     

68例微创切口小儿心内直视手术经验

目的 报告一组小儿先天性心脏病采用微创切口手术治疗的经验。方法 全组68例病儿中继发孔房间隔缺损(ASD)31例，室间隔缺损(VSD)32例，右室双腔心(DCRV)2例，肺动脉狭窄(PS)3例。右前外侧小切口不停跳、不阻断主动脉心内直视手术29例，胸部正中小切口39例。结果 除1例因术中显露差改作常规切口，全组68例无手术死亡，无严重并发症。病儿术后恢复快，平均住院时间8天。结论 微创切口小儿心内直视手术创伤小、美观、痛苦少、恢复快、住院时间短、安全有效，尤其适应于继发孔房缺及膜部或膜周室缺修补手术。     

False aneurysm of the right ventricle due to endocarditis in a child

Summary An eight-year-old boy with supravalvular pulmonic stenosis, supravalvular aortic stenosis, and ventricular septal defect developedStaphylococcus aureus endocarditis. The infection was complicated by formation of a false aneurysm of the right ventricular outflow tract, which was demonstrated by contrast echocardiogram. Surgical treatment was successful. This is a unique case of false aneurysm of the outflow tract of the right ventricle, because it is secondary to endocarditis without known previous trauma to the right ventricular wall.     

Anterolateral muscle bundle of the left ventricle in atrioventricular septal defect: Left ventricular outflow tract and subaortic stenosis

Summary The anatomy of the left ventricular outflow tract (LVOT) in 77 hearts with atrioventricular septal defect (AVSD), 36 with a separate AV orifice and 41 with a common AV-orifice, were investigated.In all specimens, an anterolateral muscle bundle of the left ventricle was identified between the superior bridging leaflet and the left coronary aortic cusp. It displaced the attachment of the superior bridging leaflet, resulting in its clockwise rotation. The muscle bundle frequently bulged into the LVOT, but was never prominent enough to have caused significant subaortic stenosis.Measurement of the LVOT aortic ratio was possible in 54 hearts and ranged from 36–100%. In 23 cases (43%), there was mild to moderate subaortic narrowing with a ratio ranging from 53–88%. In six cases (11%), unequivocal subaortic stenosis was present, mainly in AVSD with separate AV orifices (five of six) and iatrogenic in one case with surgically corrected complete defect.A decreased ratio was mainly due to decreased anteroposterior width of the septum in the subaortic area, with anterior displacement of the superior bridging leaflet in cases with dense septal attachment of the superior bridging leaflet (i.e., in AVSD with separate AV orifices, type A complete defect with small ventricular septal defect, or surgically corrected complete defect).Significant subaortic stenosis was caused by hypertrophy of the ventricular septum in the subaortic area with anteroseptal twist in four cases, by anomalous chordal insertion of the superior bridging leaflet in one case, and iatrogenic in one case after surgical correction with left AV valve replacement in a type C complete defect. Other additional obstructive forces were an anomalous papillary muscle, a small left ventricle, and an aneurysm of the membranous septum.