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PubMed1975

Situs inversus totalis associated with subaortic and subpulmonic stenosis.

The unusual occurrence of total situs inversus and idiopathic hypertrophic subaortic stenosis with the demonstration of right and left heart dynamic obstruction in one patient is presented. The fact that the patient was known to have a rare abnormality (total situs inversus) and the presenting symptom being angina pectoris, may have obscured the diagnosis of IHSS and emphasized the value of comprehensive evaluation of patients. The documentation of right ventricular dynamic obstruction, in addition to obstruction of the left side of the heart, was facilitated by the use of simultaneous pressure recordings in the pulmonary artery and in the body of the right ventricle. The angina-like pain was secondary to left ventricular outflow obstruction, rather than to coronary disease.

Adult↗
PubMed1998

Effect of atrioventricular sequential pacing on left ventricular flow dynamics in a patient with mid-ventricular obstruction.

The effect of dual chamber atrioventricular sequential pacing on the intraventricular pressure gradient was tested using Doppler echocardiography in a patient with hypertrophic mid-ventricular obstruction. Atrioventricular sequential pacing with relatively short atrioventricular delays reduced in the left ventricular pressure gradient at the mid-ventricular level. Also, atrioventricular sequential pacing affected the degree and profile of the isovolumetric relaxation flow. These results suggest that atrioventricular sequential pacing affects both systolic and diastolic left ventricular flow dynamics in mid-ventricular obstruction.

Cardiac Pacing, Artificial↗
PubMed1998

Disopyramide: a promising new approach to the medical treatment of the hypercyanotic spell complicating tetralogy of Fallot.

Under continuous ECG and oxygen saturation (SpO2) monitoring, the following measurements were taken by Doppler echocardiography in 6 consecutive patients with tetralogy of Fallot (TF) before and after intravenous administration of disopyramide (2mg/kg): left ventricular shortening fraction (LVSF); peak velocities in the right ventricular outflow tract (RVOT); diastolic and systolic internal diameters of the right ventricular outflow tract (dRVOT, sRVOT); and systolic blood pressure. SpO2 increased (p<0.01) from 78 to 98 (89 +/- 7, mean +/- standard deviation)% to 86-99 (94 +/- 5)%. LVSF decreased (p<0.05) from 0.34-0.56 (0.42 +/- 0.08) to 0.22-0.54 (0.33 +/- 0.13). The systolic blood pressure fell slightly (p<0.05) from 68-92 (79 +/- 8) to 64-92 (71 +/- 11)mmHg. The sRVOT increased (p<0.05) from 2.1-4.8 (2.7 +/- 1.5)mm to 3.0-8.1 (4.9 +/- 2.4)mm, while RVOT peak velocity decreased (p<0.05) from 2.20-4.88 (3.70 +/- 0.97)m/sec to 2.05-4.07 (2.92 +/- 0.72)m/sec. Disopyramide alleviates hypoxia in patients of TF through its negative inotropic action on right ventricular outflow obstruction.

Blood Flow Velocity↗
PubMed1989

[Use of magnesium sulfate in hypertrophic cardiomyopathy with stenosis of the outflow route].

10 patients with hypertrophic cardiomyopathy with left ventricular outflow obstruction were intravenously given 100 mg/kg b.w. of magnesium sulphate. Significant decrease of repolarization disorders was observed in ecg recordings. Polycardiographically estimated prolonged A2-O interval significantly shortened from 188 +/- 49 to 168 +/- 45 ms. Echocardiographic examinations revealed increase of the left ventricular end-diastolic dimension from 43 +/- 6 to 45 +/- 6 mm (p less than 0.05), acceleration of the diastolic, posterior wall motion from 5.7 +/- 3 cm/s to 7.2 +/- 2 cm/s (p less than 0.01) and shortening of prolonged left ventricular isovolumetric relaxation interval from 108 +/- 15 to 94 +/- 14 ms (p less than 0.05). Intrasystolic anterior, mitral leaflet motion towards the intraventricular septum also significantly decreased. There were no changes of heart rate, blood pressure and left ventricular systolic parameters after MgSO4 administration. Obtained data indicate the dynamic nature of left ventricular diastolic function impairment and its positive modification by magnesium sulphate administration.

Adult↗
PubMed1991

[Valvulopathies (XIV). The role of percutaneous valvuloplasty in congenital valvular diseases].

The present article describes the cooperative experience Córdoba-Las Palmas since 1983 in 123 patients with congenital right or left ventricular outflow obstructions who were treated by percutaneous balloon dilatation. Seventy of them had pulmonary stenosis (63 valvular and seven supravalvular types); two had pulmonary atresia, 25 valvular aortic stenosis and 27 discrete subaortic stenosis. In patients with valvular pulmonic stenosis the gradient and the right ventricular pressure decreased significantly. No significant changes were observed in the degree of pulmonary regurgitation. One neonate died. No other major complications occurred. After a mean follow up of 3 +/- 2 years, we did not observe significant changes in the degree of pressure relief. The mean residual gradient is 26 +/- 19 mmHg. In our 21 patients out of the neonatal period with valvular aortic stenosis the gradient and the left ventricular pressure dropped significantly. Progression of aortic regurgitation in more than I grade was observed in 4 patients (19%), although only one (5%) progressed to grade III. There were no major complications in this group. After a mean follow up period of 24 +/- 17 months they persist with the obtained pressure relief and same degree of valvular competence. All 4 neonates with critical aortic stenosis had unsuccessful dilations and they died, wether after ulterior surgery or without it. In 27 patients with discrete subaortic stenosis the gradient and the left ventricular pressure decreased markedly, without significant changes in valve competence. There were no related major complications. After dilation, a broken and mobile membrane was frequently seen. At follow up (24 +/- 18 months), 6 patients (22%) had restenosis; five of them were successfully redilated. The remaining 21 patients persist with a reduced residual gradient (24 +/- 7 mmHg) and without significant changes in valve competence.

Adolescent↗
PubMed1982

Repair of total anomalous pulmonary venous connection in patients younger than 6 months old. Late postoperative hemodynamic and electrophysiologic status.

During a 31/2 year period, 11 consecutive infants with total anomalous pulmonary venous connection younger than 6 months of age underwent repair at our institution. Early mortality was 19% (one of 11) and long-term mortality 18% (two of 11), yielding an overall survival of 73%. One late death was due to bradyarrhythmia and the other was due to recurrent pulmonary venous obstruction. Postoperative catheterization in seven of eight survivors and in three additional patients disclosed normal or nearly normal pulmonary artery pressure and pulmonary arteriolar resistance. Left ventricular diastolic volume was normal before operation (n = 5) and on follow-up study (n = 9). Mild left ventricular outflow obstruction or coarctation or both developed during follow-up in two patients. Sinus node function was normal in all seven late survivors studied, and atrioventricular conduction was normal in three of four. This experience indicates that total anomalous pulmonary venous connection can be repaired early in infancy, with preservation of normal hemodynamics and electrophysiologic state.

Blood Pressure↗
PubMed2003

Echocardiographic parameters in 14 healthy English Bull Terriers.

OBJECTIVE: To determine the range of various cardiac parameters using echocardiography in apparently normal, healthy English Bull Terriers. DESIGN: Fourteen English Bull Terriers were selected for study. Cardiac auscultation of the parents of these dogs was normal. Echocardiographic examination of one parent of each animal showed: no mitral or aortic valve abnormalities; no myocardial lesions; no two dimensional evidence of fixed or dynamic left ventricular outflow tract obstruction; and no systolic aortic or left ventricular outflow tract turbulence on colour flow Doppler examination. The 14 selected dogs did not have arrhythmias or murmurs, and on echocardiographic examination had similar findings to their parents. Systolic blood pressure was measured in all dogs and they had no clinical evidence of Bull Terrier polycystic kidney disease or Bull Terrier hereditary nephritis. PROCEDURE: All dogs were auscultated and subjected to a sequential global echocardiographic assessment of the heart, including two dimensional long and short axis, and colour flow Doppler interrogation of the mitral and aortic valves. Dimensional measurements, including those from the left atrium, aortic annulus and left ventricle, were taken from a right parasternal window, and derived values such as fractional shortening, stroke volume and left atrial to aortic annulus ratio were calculated. Peak systolic aortic velocity was measured from the left parasternal window using two dimensional-guided pulsed wave Doppler with angle correction. Systolic blood pressure was measured using a Doppler monitor. The absence of Bull Terrier polycystic kidney disease was determined using renal ultrasonography, and of Bull Terrier hereditary nephritis using urinary protein to creatinine ratio. RESULTS: These 14 dogs had greater left ventricular wall thickness and smaller aortic root diameters than those reported as normal for other breeds of comparable body size. Left atrial dimensions were also larger, however this may have been due to the "maximising" method of measurement. These apparently normal English Bull Terriers also had higher aortic velocities than those reported for other breeds, possibly due to a smaller aortic root diameter or other anatomic substrate of the left ventricular outflow tract, lower systemic vascular resistance, or breed-specific "normal" left ventricular hypertrophy. While these dogs were selected to be as close to normal as possible, the breed may have a particular anatomy that produces abnormal left ventricular echocardiographic parameters. CONCLUSION: These echocardiographic parameters may be used to diagnose left ventricular outflow tract obstruction and left ventricular hypertrophy, and inaccurate diagnoses may result if breed-specific values are not used.

Animals↗
PubMed1981

Cardiopulmonary bypass in the presence of uncorrected coarctation.

We performed correction of intracardiac lesions in 10 patients with coarctation. All cardiovascular lesions were corrected except coarctation. Six patients had a large left-to-right shunt though a ventricular septal defect (VSD), two patients had left ventricular outflow obstruction, two patients had mitral insufficiency, and one patient had mitral stenosis. Arterial perfusion was through the ascending aorta. Urine output was 6.0 +/- 1.5 cc/kg/hr during cardiopulmonary bypass. There was no morbidity related to cardiopulmonary bypass or inadequate perfusion of the lower body, and left ventricular failure did not occur. One patient with Taussig-Bing abnormality and pulmonary vascular disease died postoperatively of right ventricular failure. Intracardiac repair with aortic perfusion in the presence of coarctation is a safe alternative to coarctation repair or combined procedures.

Aortic Coarctation↗
PubMed1998

Primary repair is superior to initial palliation in children with atrioventricular septal defect and tetralogy of Fallot.

OBJECTIVE: The objective was to explore the best management algorithm for atrioventricular septal defect in conjunction with tetralogy of Fallot. METHODS: We reviewed the cases of 38 children referred to our division (March 1981-August 1997) who had atrioventricular septal defect associated with tetralogy of Fallot; 32 (84%) had Down syndrome. Twenty-one received initial palliation with a systemic-to-pulmonary artery shunt; of these, 2 (9.5%) died before complete repair. Thirty-one children underwent complete repair; 14 of these (45%) had undergone initial palliation (mean age at shunt 20 +/- 24 months). Right ventricular outflow obstruction was relieved by a transannular patch in 22 (71%); 14 (64% of 22) had a monocuspid valve inserted. Four required an infundibular patch. RESULTS: Two children (6.4%) died early after repair; 1 had undergone previous palliation. Patients with palliation underwent repair at an older age (78 vs 36 months), required longer ventilatory support (8 vs 4 days) and inotropic support (8 vs 4 days), and had longer intensive care stays (11 vs 6 days) and hospital stays (24 vs 15 days). Eleven children (35%) underwent reoperation, 7 (58%) for right ventricular outflow reconstruction and pulmonary arterioplasty. Reoperation was more frequent in the palliation group than in the primary operation group (64% vs 12%). The single late death was related to a reoperation in the palliation group. CONCLUSIONS: Atrioventricular septal defect with tetralogy of Fallot can be repaired with a low mortality rate. Initial palliation with a shunt resulted in a more complex postoperative course and a higher reoperative rate. Primary repair is superior to initial palliation with later repair.

Cardiac Surgical Procedures↗
PubMed2003

Cause of normal pulmonic velocity in fetal tetralogy of Fallot.

In 10 fetuses with tetralogy of Fallot, 9 lacked high velocity across the obstructed right ventricular outflow tract, which we conclude was due to the parallel flow circulations created by the combination of ventricular septal defect and arterial duct. The normally high resistance in the fetal pulmonary vascular system substantially exceeds the resistance of the systemic vasculature, resulting in the maintenance of right-to-left ductal flow in all 10 fetuses who had only moderate pulmonary obstruction.

Blood Flow Velocity↗
PubMed2006

Isolated subvalvular pulmonary stenosis: depiction at whole heart magnetic resonance imaging.

Isolated subvalvular pulmonary stenosis is a rare condition and its morphological evaluation is obscure. Whole heart magnetic resonance imaging (MRI) is a new, totally non-invasive technique which allows three-dimensional comprehension of the cardiac structure. We describe a patient with isolated subvalvular pulmonary stenosis, in whom whole heart MRI was useful to detect and evaluate the right ventricular outflow obstruction.

Humans↗
PubMed1985

Cardiac output in infants of insulin-dependent diabetic mothers.

The purpose of our study was to quantitate left ventricular outflow obstruction in the infant of the diabetic mother (IDM) through estimation of cardiac output by pulsed Doppler ultrasound. We evaluated 42 IDMs (White class B, C, or D) and compared them with two control groups, one similar in birth weight and the other similar in gestational age. Ventricular septal hypertrophy was found in 18 (43%) of 42 of the IDMs, but in none of the control infants. Morbidity increased with advancing septal thickness. None had hyaline membrane disease. Twenty-nine IDMs were asymptomatic, 11 of whom had septal hypertrophy. Thirteen IDMs developed congestive heart failure, seven of whom had septal hypertrophy. Cardiac output per kilogram diminished linearly with increasing septal thickness (r = -0.78, P less than 0.001) secondary to reduced stroke volumes at comparable heart rates. The IDMs had higher left atrial/aortic ratios and greater right ventricular systolic time intervals than the control infants. We conclude that cardiac output is significantly reduced in IDMs with septal hypertrophy. This reduction is secondary to reduced stroke volume and is directly related to the degree of septal hypertrophy.

Birth Weight↗
PubMed1988

Ventriculocoronary connections in hypoplastic right heart syndrome: autopsy serial section study of six cases.

Myocardial sinusoids communicating with the coronary systems occur in pulmonary atresia with intact ventricular septum. To test the hypothesis that the extent of ventriculocoronary connections correlates with the degree of right ventricular outflow obstruction as evidenced by clinical, angiographic and gross anatomic findings, a serial section study of six human autopsy hearts representing a spectrum of hypoplastic right heart was undertaken. Slides were evaluated for the presence and extent of ventriculocoronary connections, associated developmental abnormalities and secondary changes in the ventricular walls. Whereas extensive blind-ended deep sinusoids were a feature of all five cases with unrelieved obstruction, ventriculocoronary connections were identified in three. Changes that suggested ongoing remodeling provide new evidence for the postnatal temporal evolution of these anomalous communications. The regional distribution of myofiber disarray in hypoplastic right heart supports the concept that vascularization parallels myocardial organization in the developing human heart.

Coronary Vessels↗
PubMedDate not supplied

Aneurysm of the membranous ventricular septum and accessory valvular tissue causing obstruction of the pulmonary outflow tract in corrected transposition.

Aneurysm of the membranous portion of the ventricular septum and accessory valvular tissue are two infrequent congenital lesions. This case illustrates a rare association of aneurysm of the membranous septum and accessory valvular tissue originating from the mitral valve, causing right-sided ventricular outflow tract obstruction in a patient with corrected transposition. Multiplane transesophageal echocardiography allowed for determination of the dual nature of the obstruction.

Adult↗
PubMed1985

Surgery for discrete subvalvular aortic stenosis: actuarial survival, hemodynamic results, and acquired aortic regurgitation.

Discrete membranous subaortic stenosis (DMSS) accounts for 8 to 30% of congenital left ventricular outflow obstruction. The immediate effectiveness of surgical resection of this discrete obstructing membrane has been well documented, but little has appeared regarding late clinical and hemodynamic follow-up. Fifty-three patients with DMSS underwent operation at our institution from 1957 to 1983. Most (78%) were symptomatic, 79% had left ventricular hypertrophy (LVH) by electrocardiogram, and 92% had roentgenographic evidence of cardiomegaly preoperatively. Catheterization revealed a mean preoperative left ventricular-aortic gradient of 89 mm Hg. Twenty-eight patients had associated aortic insufficiency (AI) on the initial aortogram. Seven patients acquired AI in the interim between the first and second preoperative catheterization. Our patients have been followed for up to 12 years postoperatively. There have been 2 early and 3 late deaths. (Actuarial analysis revealed 5- and 10-year survival of 95% and 83%, respectively.) Seventy-one percent of the previously symptomatic patients noted relief of their preoperative complaints, and 45% of those with LVH had a regression in voltage. Cardiomegaly as determined by chest roentgenogram decreased in 45%. The left ventricular-aortic gradient fell to an average of 35 mm Hg a year postoperatively. Surgical treatment of congenital subvalvular aortic stenosis is effective in reducing the preoperative symptoms and the left ventricular-aortic gradient. It appears that DMSS causes AI.

Actuarial Analysis↗
PubMed1997

Hemodynamic performance of small aortic valve bioprostheses: is there a difference?

BACKGROUND: There is the potential for left ventricular outflow obstruction when small aortic valve bioprostheses are employed in normal-sized or large adults. It has been hoped that bovine pericardial valves would improve hemodynamic performance in the smaller tissue valve sizes. METHODS: To determine in vivo hemodynamic performance of heterograft aortic valve prostheses, we analyzed echocardiographic data from patients receiving 21- or 23-mm Carpentier-Edwards pericardial, Medtronic Intact, and Carpentier-Edwards porcine bioprostheses. In addition, data from 19-mm Carpentier-Edwards pericardial valves were included for comparison of hemodynamic performance between valve sizes. Doppler echocardiography was performed in 151 patients within 2 weeks of operation. Left ventricular outflow gradient was derived from continuous Doppler measurements of flow velocity, and effective orifice area was calculated by the continuity equation. RESULTS: There were statistically significant differences in hemodynamic performance of different sized prostheses for each valve type (effective orifice area, p < 0.01; valvular gradient, p < 0.03). There were, however, no significant differences in effective orifice area or mean gradient for different valve types within each size category. CONCLUSIONS: The in vivo hemodynamic performance of these three different aortic valve heterograft bioprostheses is similar. Patient-prosthesis mismatch with heterograft prostheses, as demonstrated by the indexed effective orifice area can be avoided by appropriate sizing and use of annular enlarging techniques when necessary.

Adult↗
PubMedDate not supplied

A case report of surgical septal myectomy of hypertrophic cardiomyopathy with concomitant left ventricular outflow tract and mid-ventricular obstructions.

A 27-year-old female presented with dyspnea, fatigue, and exertional angina is found to have hypertrophic cardiomyopathy with marked hypertrophy of the papillary muscles, apex, septum, and lateral wall of the left ventricle. Also, small left ventricular cavity and systolic anterior movement of anterior mitral leaflet were observed at the echocardiography. The Doppler echocardiography revealed severe peak gradients at the left ventricle outflow tract (105 mmHg) and mid-ventricle (80 mmHg). At the operation, septal myectomy and anterior papillary muscle resection in addition to mitral valve replacement was performed. Surgical treatment gave an excellent clinical result. Control Doppler echocardiograms revealed no left ventricular outflow tract gradient, although mid-ventricular gradient was persistent. The good results were still present 18 months after the operation.

Adult↗
PubMedDate not supplied

Systolic aortic valve compression from partial dehiscence of an aortic valve homograft.

Implantation of valve prostheses provide improvement of symptoms and prolongation of life in selected patients with valvular heart disease. Meticulous follow-up of patients after valve surgery is essential as complications of valve failure, valve dehiscence, valve thrombosis, and infection may occur. The major mode of failure of aortic valve homografts is valve regurgitation, which is readily detected by physical examination. We report a case of left ventricular outflow obstruction after implantation of an aortic valve homograft.

Adult↗