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The infundibular septum in normal hearts and in hearts with isolated ventricular septal defect. A comparison between Japanese and Dutch hearts.

The observation that subpulmonary or doubly committed subarterial ventricular septal defects occur with a high frequency among orientals as compared to westerners has led to the hypothesis that a racial difference exists with regard to normal and abnormal development of the embryonic conal septum. In an attempt to contribute to this concept we have studied 24 normal hearts and 70 hearts with a perimembranous ventricular septal defect from patients of Japanese and Dutch origin. The length of the infundibular septum and the length of the right ventricle were not statistically different between the 2 groups. These findings suggest that in normal hearts and in hearts with perimembranous ventricular septal defects no racial differences exist with respect to the role of the embryonic conal septum in contributing to the definitive infundibular septum. The results indicate that subpulmonary or doubly committed subarterial defects may have a different origin from that of the usual perimembranous defect.

Asian People↗

The surgical anatomy of the left ventricular outflow tract in hearts with ventricular septal defect and aortic arch obstruction.

BACKGROUND: Profound understanding of the left ventricular outflow tract (LVOT) anatomy is crucial to improve surgical results in patients with aortic arch obstruction, ventricular septal defect, and subaortic stenosis. METHODS: We studied the morphology of the LVOT in 32 postmortem hearts with aortic arch obstruction and a ventricular septal defect. In case of subaortic obstruction, the length of the subaortic muscular component was measured anteriorly and posteriorly within the left ventricle. RESULTS: Seven of the 32 hearts had no subaortic stenosis. Nine had aortic override, which caused LVOT narrowing. Sixteen hearts contained a subaortic shelf, downstream to the ventricular septal defect, which deviated into the left ventricle in 15. In 10 of these the shelf was muscular; in 6 it was a fibrous ridge. In cases with a muscular shelf, the posterior part was significantly shorter than the anterior part (p < 0.004). In 9 hearts the LVOT was further narrowed because of the abnormal relationship between the mitral valve and the subaortic shelf. CONCLUSIONS: The present study confirms the complexity of LVOT stenosis in aortic arch obstruction and ventricular septal defect and provides a better understanding of the options to achieve surgical relief.

Aorta, Thoracic↗

Growth parameters and endocrine function in relation to echocardiographic parameters in children with ventricular septal defect without heart failure.

To determine the effect of ventricular function, size of ventricular septal defect (VSD), and endocrine function on linear growth in children with VSD, we studied 88 children with VSD over a period of 1 year. Growth was assessed by determining the height standard deviation scores (HtSDS) and growth velocity (GV) every 4 months. Two hundred age-matched normal children served as controls for the growth data. Endocrine evaluation was performed in 30 randomly selected children with VSD, and 20 age-matched children with constitutional delay of growth (CSS). Growth hormone (GH) response to clonidine provocation was evaluated and circulating free thyroxine (FT4) and insulin-like growth factor-I (IGF-I) concentrations measured. Echocardiographic evaluation of the different cardiac parameters including shunt size and shunt fraction (Qp/Qs) was performed using a colour-coded echodoppler. The HtSDS, body mass index (BMI), and mid-arm circumference (MAC) of children with VSD were significantly decreased compared to those for the normal control group. The dietary intake evaluated by the recall method, appeared to be adequate in the majority of these children (83/88). IGF-I concentrations were reduced in children with VSD (87.5 +/- 29 ng/ml) versus normal age-matched children (169 +/- 42 ng/ml). Basal and clonidine-stimulated GH concentrations were significantly higher in children with VSD (4.6 +/- 2.1 microg/l and 28.8 +/- 7.9 microg/l respectively) versus controls (17.8 +/- 4.2 microg/l). In these patients (n = 88) the HtSDS was correlated negatively with the size of the shunt (r = -0.793, p < 0.001), shunt fraction (Qp/Qs) (r = -0.76, p < 0.001), pulmonary mean gradient (r = -0.4, p = 0.006), and pulmonary maximum velocity (r = -0.32, p = 0.02). Growth velocity (GV) was correlated negatively with pulmonary maximum gradient (r = -0.3, p = 0.02), pulmonary maximum velocity (r = -0.37, p = 0.007), and pulmonary stroke volume (Qp) (r = -0.345, p = 0.01). The BMI and IGF-I concentrations were correlated significantly with the size of the shunt (r = -0.453, p < 0.01), Qp/Qs (r = -0.432, p < 0.01), HtSDS (r = 0.565, p < 0.01), and BMI (r = 0.435, p < 0.01). It appears that in patients with VSD, the size of the left-to-right shunt and the abnormal hemodynamics in the pulmonary circulation are important factors in the etiology of impaired growth. It is suggested that the hypermetabolic status of these patients compromise nutrition and this decreases IGF-I synthesis with subsequent slowing of linear growth and weight gain.

Body Mass Index↗

Identification of differentially expressed genes in human heart with ventricular septal defect using suppression subtractive hybridization.

Ventricular septal defect (VSD) accounts for the largest number of birth congenital heart defects in human, but the genetic programs that control ventricular septation are poorly understood. To identify differentially expressed genes between ventricular septal defect and normal ventricular septum myocardium, we have undertaken suppression subtractive hybridization (SSH) and generated reciprocal cDNA collections of representative mRNAs specific to human heart with ventricular septal defect versus normal control. Following SSH, 1378 clones were sequenced and found to derive from 551 different genes. These predominately expressed genes included genes involved in energy metabolism, cell cycle and growth, cytoskeleton and cell adhesion, LIM protein, zinc finger protein, and development. It is anticipated that further study of genes identified will provide insights into their specific roles in the etiology of VSD, even in cardiac development, aging, and disease.

Adult↗

Surgical technique to reduce the risks of heart block following closure of ventricular septal defect in atrioventricular discordance.

Traumatic heart block remains a major concern after ventricular septal defect (VSD) closure in patients with atrioventricular discordance. A technique of closing the VSD, placing the suture line on the morphologically right side of the septum without opening the systemic ventricle, is described. This was used in 13 consecutive patients. The VSD was closed through the right atrium in eight patients, the left ventricle in three patients, the right atrium and the left ventricle in one patient, and through the right ventricle in one patient with atrioventricular disordance and ventriculo-arterial concordance. All patients were in sinus rhythm preoperatively, two exhibited atrioventricular dissociation before intracardiac manipulation began, and 11 patients were in sinus rhythm postoperatively. No major arrhythmia could be attributed to the closure of the VSD.

Adolescent↗

[Ebstein's anomaly of the heart with ventricular septal defect at age 65].

A postmortem in a woman of 65 revealed Ebstein's anomaly of the heart with ventricular septum defect. The defect had been closed from the right side by the septal cups of the tricuspid valve coalescing with its edges. Literary sources refer to only 6 cases of Ebstein's anomaly in subjects older than our patient. The simultaneous presence of spontaneously closed defect of ventricular septum makes it an entirely exceptional case.

Aged↗

Surgical correction of cardiogenic shock.

Cardiogenic shock as a result of myocardial infarction demands an aggressive diagnostic and therapeutic approach to improve survival. First, the patient likely to develop shock should be identified early in his course so that interventions to reduce or contain infarct progression and thus prevent shock can be instituted. If the patient's condition deteriorates, prompt precise diagnosis of the cause of the hemodyanamic impairment must be made at the bedside with Swan-Ganz right heart catheterization and echocardiography, and, if potentially surgically correctable disease is present, left heart catheterization with coronary angiography in the catheterization laboratory should be done. Surgical repair of anatomic disruptions of the heart ventricular septal defect, or mitral valve rupture is frequently life-saving. At present, operative intervention for shock secondary to massive myocardial infarction alone remains a challenge.

Aortic Aneurysm↗

The conduction system in double outlet right ventricle with subpulmonic ventricular septal defect and related hearts (the Taussig-Bing group).

The Taussig-Bind group of hearts is that group in which the aorta emerges completely from the right ventricle, unrelated to the position of the ventricular septal defect (VSD), while the pulmonary trunk emerges at varying degrees from the right ventricle related to the location of the VSD. Several types of surgical procedures involve the VSD in these hearts. The conduction systems in six of these hearts were therefore studied. The atrioventricular (A-V) bundle lies in the inferior (posterior) wall of the defect on the left side beneath the summit of the ventricular septum. In some cases, the bundle may be proximal to the defect, thus lying intramuscularly, and only the right and left bundle branches are related to the defect. The pattern od development of the atrioventricular (A-V) node, bundle and bundle branches is related primarily to the development of the endocardial cushions and posterior ventricular septum, and not to that of the absorption of the bulbus.

Aorta↗

Comparison of dopamine and dobutamine in patients requiring postoperative circulatory support.

We compared dopamine and dobutamine following cardiac surgery in a sequential cross-over study of 9 patients who required inotropic support after volume replenishment. Seven patients had mitral valve replacement, 2 with simultaneous coronary revascularization (CABG), and 1 each with simultaneous tricuspid annuloplasty and aortic valve replacement; 1 had isolated CABG and 1 had repair of a postinfarction ventricular septal defect. Heart rate, right and left atrial pressures, pulmonary and mean arterial pressure, systemic and pulmonary vascular resistance, and cardiac index were measured and calculated. Inotropic support with dopamine or dobutamine was begun for treatment of low cardiac index within 12 hours postoperatively. Measurements were made after 20 minutes on either agent at 5-10 micrograms/kg per min when cardiac output and general clinical state were stable. Each patient was then switched to the other agent at approximately the same dose rate, titrating the dosage to the same cardiac output, and repeat measurements were made at 20 minutes when again stable. Similar dosages of dopamine (6.2 +/- 1.7 micrograms/kg/min) and dobutamine (6.7 +/- 2.5 micrograms/kg/min) produced equivalent heart rate (103 +/- 19 vs. 102 +/- 13 beats/min, p = NS), cardiac index (2.8 +/- 1.1 vs. 2.9 +/- 1.2 l/min/m2, p = NS), and pulmonary arterial pressure and vascular resistance. Mean systemic arterial pressure was significantly lower with dobutamine (59 +/- 5 vs. 67 +/- 7 mmHg, p less than 0.05) as were mean left (14 +/- 5 vs. 18 +/- 6 mmHg, p less than 0.0001) and right (9 +/- 2 vs. 11 +/- 2 mmHg, p less than 0.05) arterial pressures.(ABSTRACT TRUNCATED AT 250 WORDS)

Aged↗

[Comparison of the late hemodynamics following corrective surgery of congenital heart diseases: ventricular septal defect, tetralogy of Fallot, Mustard operation and Fontan operation].

Late hemodynamics following corrective surgery for congenital heart diseases were evaluated in 65 patients. The patients were divided into 4 groups due to the operative procedures including closure of ventricular septal defect (VSD group = 15 patients), repair of tetralogy of Fallot (TOF group = 20), Mustard operation (Mustard group = 19) and Fontan operation (Fontan group = 11). The postoperative period was 3.8 +/- 3.8, 1.0 +/- 0.1, 5.7 +/- 4.3 and 3.2 +/- 4.9 years in VSD, TOF, Mustard and Fontan groups, respectively. Postoperative pulmonary to systemic arterial pressure ratio in VSD group significantly decreased to 0.38 +/- 0.13 from the preoperative value of 0.97 +/- 0.04. Pulmonary vascular resistance (Rp) in VSD group significantly decreased from 4.88 +/- 2.40 to 2.56 +/- 0.75 U.m2 and Rp in Fontan group significantly increased from 1.63 +/- 0.70 to 3.66 +/- 0.47 U.m2 postoperatively. Postoperative cardiac indices in VSD, TOF, Mustard and Fontan groups were 4.30 +/- 1.34, 4.28 +/- 0.72, 3.83 +/- 1.38 and 3.48 +/- 1.38 l/min/m2, respectively, without significant differences. Ejection fraction (EF) of the systemic ventricle (left ventricle) in VSD, TOF and Fontan groups were 0.66 +/- 0.06, 0.65 +/- 0.07 and 0.63 +/- 0.08, respectively. In Mustard group, EF of the systemic ventricle (right ventricle) was 0.56 +/- 0.12 and significantly less than left ventricular EF in the other 3 groups. The hemodynamic characteristics after these operative procedures should be considered for patient management.

Cardiac Surgical Procedures↗