Giant right atrium and subvalvular pulmonary stenosis: A case report of an interesting combination.

A 20-year-old Congolese woman presented with presyncope, dyspnea, and anasarca. Past medical history was unremarkable. Echocardiography revealed a rare combination of giant right atrium (RA), a dilated and hypertrophied right ventricle, subvalvular pulmonary stenosis (subPS), severe tricuspid regurgitation (TR), pericardial effusion and what appeared to be a spontaneously closed ventricular septal defect (VSD). Cardiac Magnetic Resonance and Cardiac Computed Tomography confirmed the findings excluding the presence of intra-cardiac and extra-cardiac shunt and other associated congenital anomalies. The patient underwent subPS resection, right atrioplasty, and tricuspid annuloplasty. Multimodality approach facilitated the detection of the abnormalities and provided clarity when determining the optimal surgical strategy.

Primary pulmonic infundibular stenosis in 12 cats: natural history and the effects of balloon valvuloplasty.

OBJECTIVES: To evaluate the natural history of primary pulmonic infundibular stenosis in cats and the effects of balloon valvuloplasty. BACKGROUND: Primary pulmonic infundibular stenosis is an uncommon congenital defect in cats. The natural history of the disease has not been described. Information regarding balloon valvuloplasty in the cat is limited. ANIMALS: Records between January 1, 1999 and December 31, 2005 were reviewed and cats with a confirmed echocardiographic diagnosis of primary pulmonic infundibular stenosis, a complete medical history, and no evidence of significant systemic disease were identified. METHODS: Echocardiographic, electrocardiographic, and radiographic findings are described. The natural history of those with severe disease was compared to those with mild to moderate disease. Balloon valvuloplasty was performed in six of the cats. The technique used is described. RESULTS: A stenotic gradient >or=70 mmHg and a right ventricular outflow tract (measured at the level of the stenosis) to pulmonary valve annulus ratio of

Subpulmonary and subaortic ridges in doubly committed subarterial ventricular septal defect: an echocardiographic study.

BACKGROUND: Certain conditions, including discrete subaortic stenosis and right ventricular outflow tract obstruction are well-known associations with ventricular septal defect. However, the association of discrete type subpulmonary and subaortic stenosis with ventricular septal defect has not been described to date. PATIENTS: In this report we present our experience in nine patients with doubly committed subarterial ventricular septal defect in whom associated discrete subpulmonary and subaortic stenosis were diagnosed by two-dimensional echocardiography. The mean age of the patients was 5.7 years, and eight of them were male. Two patients had additional hypertrophied anomalous muscle bundles with pressure gradients across the right ventricular outflow tract. Aortic valve prolapse was detected in two patients with one of them having mild aortic regurgitation. INTERVENTION: Cardiac catheterization was performed in four patients, and three underwent surgery. Doubly committed subarterial ventricular septal defect was closed, and subpulmonary and subaortic ridges were resected in each patient. Histological findings were similar for both ridges, which were composed of thin, short and irregularly arranged elastic fibres covered by endothelial cells. RESULTS: The finding of fibrous ridge on both sides of the doubly committed ventricular septal defect, where the turbulence is likely to be maximum, and the histopathological similarities of subpulmonary and subaortic ridges, may suggest a common mechanism for the development of fibrous ridges. CONCLUSION: Serial echocardiographic assessment of patients with doubly committed ventricular septal defect is recommended.

Infundibular pulmonic stenosis with intact ventricular septum: a report of 15 surgically corrected patients.

Infundibular pulmonic stenosis with intact ventricular septum of primary origin is an uncommon condition. We report 15 such patients (nine males and six females, aged 7-36 years) who had undergone surgical correction for the anomaly during the period between 1975 and 1992. The occurrence of this clinical setting represents 0.19% (15/7826) of all cardiac operations and 0.46% (15/3222) of congenital heart diseases undergoing surgical correction during that period of time. The lesion was of discrete fibromuscular hypertrophy of the infundibulum in all 15 patients. The presenting symptoms of most patients were exertional dyspnea and syncope; however, five patients with severe obstruction were asymptomatic. The peak systolic pressure gradient across the infundibulum ranged from 71 to 230 mmHg. There was only one operative death; the remainder had remained well following the surgery over a mean follow-up period of 35 months. Surgical correction for infundibular pulmonic stenosis is rewarding in the absence of heart failure.

Conal anatomy in 119 patients with d-loop transposition of the great arteries and ventricular septal defect: an echocardiographic and pathologic study.

OBJECTIVES: We sought to study the range of conal morphology in transposition of the great arteries with ventricular septal defect and their embryologic and surgical implications. BACKGROUND: Conal anatomy in transposition of the great arteries and ventricular septal defect is variable and might affect surgical repair. METHODS: Conal anatomy was explored using two-dimensional echocardiography in 119 patients with transposition of the great arteries and a large ventricular septal defect who presented between 1984 and 1991. The influence of conal anatomy on surgical technique was determined by review of the operative reports. Specimens of transposition of the great arteries with unusual conal anatomy were selected from the Cardiac Registry for comparison with the echocardiograms. RESULTS: One hundred five patients (88.2%) had subaortic conus only with no subpulmonary conus (Group 1). Subarterial conus was present bilaterally in eight patients (6.7%) (Group 2). Four patients (3.4%) had only subpulmonary conus with no (or minimal) subaortic conus (Group 3). Among these four patients, the aorta was posterior to the pulmonary artery in one patient, side by side relative to the pulmonary artery in two patients and slightly anterior in the fourth patient. Subarterial conus was absent bilaterally in two patients (1.7%) (Group 4); the aorta was slightly posterior in one and side by side with the pulmonary artery in the other. CONCLUSIONS: This variability of conal anatomy in transposition of the great arteries with ventricular septal defect implies four mechanisms by which transposition can occur. The conal anatomy appeared to affect surgical repair in Groups 1 and 2 insofar as it influenced ventricular outflow tract obstruction. In Groups 3 and 4, an arterial switch operation was performed in four of the six patients. The posterior location of the aorta obviated the need for the Lecompte maneuver in two of these four patients. In the remaining two cases in Groups 3 and 4, the condition was repaired by directing the left ventricular outflow across the ventricular septal defect to the aorta using a patch, with or without placement of a conduit from the right ventricle to the pulmonary artery.

Fetal cardiovascular cross-sectional morphology of tetralogy of Fallot in rats.

In order to clarify cross-sectional morphology of tetralogy of Fallot in the fetus, 202 rat fetuses with tetralogy of Fallot induced by maternal administration of bis-diamine were studied using cesarean section, the whole body freezing method and by photographing cross sections of the fetal heart on a freezing microtome. Some characteristic features such as large subaortic ventricular septal defects, aortic overriding over the ventricular septum, aortomitral fibrous continuity, and narrow infundibulum and pulmonary valve were found. Moreover, three subtypes were noticed. In classic tetralogy of Fallot with stenotic infundibulum, the size of the pulmonary arteries was smaller in proportion to the infundibular stenosis. In the second type with absent infundibular septum and severe valvular pulmonary stenosis, the pulmonary arteries were most hypoplastic. In the third type with absent pulmonary valve, the pulmonary arteries were dilated forming aneurysms. In the last type only, ventricles were dilated and grossly hypertrophic.

[A case report of isolated infundibular pulmonary stenosis with pouch of the infundibular chamber].

We experienced a surgical correction of isolated infundibular pulmonary stenosis (IIPS) of a 50-year-old male patient. The infundibular tissue was resected by the transpulmonary approach, and no trace of ventricular septal defect was found. There was a couple of pouch-like structure in the infundibular chamber of the right ventricle. The etiology of this structure was likely to be related turbulence as blood was ejected from the right ventricle to the infundibular chamber. Surgical correction of IIPS over 50 years old had been rarely reported. In addition, it was the first case report of IIPS with pouch of the infundibular chamber.

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

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.

An autopsy case of sudden unexpected death due to atherosclerotic coronary heart disease associated with simple right ventricular outflow tract stenosis.

A case of a 35-year-old male who died suddenly after a blow on the chest by his opponent during a quarrel. From both the clinical and autoptical examinations there was no evidence of trauma found anywhere, even in the chest. The heart was the only one of all the internal organs whereon significant pathologic changes were observed during autopsy. It's lesions were as follows: (I) The right ventricle was greatly enlarged simply due to the simple right ventricular outflow tract stenosis (SRVOTS); the heart weight was 587 g. (II) All the main trunks of coronary arteries were involved by atherosclerosis. In addition, the appearance of the lumen at the proximal end of the left anterior descending artery narrowed by an atheromatous plaque (grade II) was completely occluded by a newly formed thrombus. The authors believed that both the coronary atherosclerotic changes and the enlarged heart due to the SRVOTS played a role in the pathogenesis of death (Kelberlus, H.E. and Wellens, H.J.J., Sudden Death, Developments in Cardiovascular Medicine, Vol. 4, Martinys Nijhoff Publishers, London, 1980, pp. 34-48; Jinming, Qin, A study of cardiac sudden death (51 cases) on the clinical pathologic view, Tianjin Med., 8 (1980) 458-461). The quarrel and blow were the disposing factor.

Architecture of the ventricular mass and atrioventricular valves in complete transposition with intact septum compared with the normal: I. The left ventricle, mitral valve, and interventricular septum.

Venous correction for complete transposition with intact ventricular septum remains the preferred surgical option in most centers (Mustard or Senning procedures). The long-term function of the morphologically right ventricle and tricuspid valve, which continue to sustain the systemic circulation thereafter, remains a matter of concern. Some centers have been performing arterial redirection ("arterial switch") as the surgical treatment of transposition. Consequently, the status of the left ventricle and mitral valve is of equal interest. Yet, detailed morphological studies of the ventricles, atrioventricular valves, and their tension apparatus in "simple" complete transposition are lacking. We have examined the morphology of the left ventricle and the spatial relationships of the component parts of the interventricular septum (part I) as well as the morphology of the right ventricle and the tricuspid valve (part II). A total of 77 specimens with complete transposition and intact ventricular septum were examined and compared with 50 normal hearts. Consistent deformities were noted in relation to the orientation of the ventricular septum. The ratios of ventricular outlet to the inlet lengths were increased in both ventricles and the posterior arterial valve was abnormally related to the atrioventricular valves. These anomalies are similar in some respects to those found in atrioventricular septal defects. Their significance in relation to long-term function remains unclear.

Echocardiographic evaluation of left ventricular outflow tract obstruction in complete transposition of the great arteries.

Subpulmonic stenosis in complete d-transposition of the great arteries (d-TGA) is a frequently associated malformation, the precise diagnosis of which is essential for optimal medical and surgical treatment. Sixteen patients with d-TGA and subpulmonic stenosis have been studied by M-mode and two-dimensional (2DE) echocardiography and cardiac catheterization. Dynamic obstruction was found in six patients and fixed stenosis in 10. Systolic anterior motion of the mitral valve without fixed obstruction of the left ventricular outflow tract (LVOT) was present in patients with dynamic stenosis. Measurements of left ventricular end-diastolic posterior wall thickness to minor semiaxis ratio correlated well (p less than 0.001) with the pressure gradient across the LVOT. Various types of anatomic fixed obstruction are described. M-mode echocardiography provides assessment of dynamic obstruction but does not allow quantitative evaluation of the length of the narrowed segment. The latter can be achieved by 2DE, which offers improved definition of different anatomic types.