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PubMed1992

Obstruction of right ventricular outflow tract caused by intracavitary metastatic disease: analysis of 14 cases.

Obstruction of the right ventricular outflow tract from metastatic disease is rare. Eleven previous case reports and three new cases are presented. Two tumor types (pancreas and breast), not previously associated with right ventricular outflow tract obstruction, are included. Congestive symptoms, systolic murmur and right axis deviation or right bundle branch conduction abnormality were universal features. Echocardiography is valuable in the delineation of metastatic cardiac involvement and the detection of intracardiac gradients. Adverse hemodynamic consequences developed in 3 of 10 patients who underwent right heart catheterization in which two died. This procedure should be performed only when absolutely necessary. Metastatic obstruction of the right ventricular outflow tract should be considered in the absence of widespread malignancy because the heart was the sole site of metastasis in 5 of 10 autopsy patients. Two patients with solitary cardiac metastasis benefited from resection of the obstructing tumor, underscoring the importance of early identification of this subgroup.

Adenocarcinoma↗
PubMed1984

Continuous wave Doppler velocimetry as an adjunct to cross sectional echocardiography in the diagnosis of critical left heart obstruction in neonates.

If cross sectional echocardiography in isolation is used to diagnose critical left ventricular outflow obstruction in neonates, false positive and false negative diagnoses may result. Continuous wave Doppler velocimetry was used to measure blood flow velocity in the ascending and descending thoracic aorta in six neonates (aged less than 6 weeks) presenting with reduced or absent peripheral pulses in order to determine the important sites of obstruction. This technique demonstrated abnormal high velocity blood flow jets (three times higher than normal) in the ascending aorta in three patients with normal descending aortic flow velocity, suggesting aortic stenosis. In the other three patients velocity in the ascending aorta was normal but high in the descending aorta, suggesting coarctation. The Doppler diagnosis was confirmed in the five patients who required surgery. Two patients had residual high velocity jets after aortic valvotomy. Both had significant pressure gradients across the aortic valve at cardiac catheterisation with good agreement between actual gradients and those predicted by the Doppler technique. Thus a combined anatomical and physiological approach using cross sectional echocardiography and continuous wave Doppler velocimetry enables accurate non-invasive definition of the site of left ventricular outflow obstruction and may obviate the need for invasive investigation in these sick neonates.

Aortic Coarctation↗
PubMed1997

Stress-induced left ventricular outflow tract obstruction: a potential cause of dyspnea in the elderly.

OBJECTIVES: We sought to identify the pattern of disturbed left ventricular physiology associated with symptom development in elderly patients with effort-induced breathlessness. BACKGROUND: Limitation of exercise tolerance by dyspnea is common in the elderly and has been ascribed to diastolic dysfunction when left ventricular cavity size and systolic function appear normal. METHODS: Dobutamine stress echocardiography was used in 30 patients (mean [+/-SD] age 70 +/- 12 years; 21 women, 9 men) with exertional dyspnea and negative exercise test results, and the values were compared with those in 15 control subjects. RESULTS: Before stress, left ventricular end-diastolic and end-systolic dimensions were reduced, fractional shortening was increased, and the basal septum was thickened (2.3 +/- 0.5 vs. 1.4 +/- 0.2 cm, p < 0.001, vs. control subjects) in the patients, but posterior wall thickness did not differ from that in control subjects. Left ventricular outflow tract diameter, measured as systolic mitral leaflet septal distance, was significantly reduced (13 +/- 4.5 vs. 18 +/- 2 mm, p < 0.001). Isovolumetric relaxation time was prolonged, and peak left ventricular minor axis lengthening rate was reduced (8.1 +/- 3.5 vs. 10.4 +/- 2.6 cm/s, p < 0.05), suggesting diastolic dysfunction. Transmitral velocities and the E/A ratio did not differ significantly. At peak stress, heart rate increased from 66 +/- 8 to 115 +/- 20 beats/min in the control subjects, but blood pressure did not change. Transmitral A wave velocity increased, but the E/A ratio did not change. Left ventricular outflow tract velocity increased from 0.8 +/- 0.1 to 2.0 +/- 0.2 m/s, and mitral leaflet septal distance decreased from 18 +/- 2 to 14 +/- 3 mm, p < 0.001. In the patients, heart rate rose from 80 +/- 12 to 132 +/- 26 beats/min and systolic blood pressure from 143 +/- 22 to 170 +/- 14 mm Hg (p < 0.001 for each), but left ventricular dimensions did not change. Peak left ventricular outflow tract velocity increased from 1.5 +/- 0.5 m/s (at rest) to 4.2 +/- 1.2 m/s; mitral leaflet septal distance fell from 13 +/- 4.5 to 2.2 +/- 1.9 mm (p < 0.001); and systolic anterior motion of mitral valve appeared in 24 patients (80%) but in none of the control subjects (p < 0.001). Measurements of diastolic function did not change. All patients developed dyspnea at peak stress, but none developed a new wall motion abnormality or mitral regurgitation. CONCLUSIONS: Although our patients fulfilled the criteria for "diastolic heart failure," diastolic dysfunction was not aggravated by pharmacologic stress. Instead, high velocities appeared in the left ventricular outflow tract and were associated with basal septal hypertrophy and systolic anterior motion of the mitral valve. Their appearance correlated closely with the development of symptoms, suggesting a potential causative link.

Aged↗
PubMed2001

Critical aortic stenosis in the neonate: a multi-institutional study of management, outcomes, and risk factors. Congenital Heart Surgeons Society.

OBJECTIVES: We sought to determine factors that would predict whether a biventricular repair or Norwood procedure pathway would give the best survival in neonates with critical aortic stenosis. METHODS: Survival and risk factors were determined with parametric time-dependent event analysis for patients undergoing either the Norwood procedure or biventricular repair, and predicted optimal pathway and survival benefit were derived from multivariable linear regression. RESULTS: From 1994 to 2000, 320 neonates with critical left ventricular outflow obstruction were entered into a prospective multi-institutional study. Patients who died without intervention (n = 19) and those with primary cardiac transplantation (n = 6) were excluded. An initial intended biventricular repair pathway was indicated in 116 patients, with survival of 70% at 5 years. An initial Norwood procedure was performed in 179 patients, with survival of 60% at 5 years. Using predictions from separate multivariable hazard models for survival with each of the 2 pathways, we determined predicted optimal pathway and survival benefit for each patient. Significant independent factors associated with greater survival benefit for the Norwood procedure versus biventricular repair included younger age at entry, lower z-score of the aortic valve and left ventricular length, higher grade of endocardial fibroelastosis, absence of important tricuspid regurgitation, and larger ascending aorta. Predicted survival benefit favored the Norwood procedure in 50% of patients who had biventricular repair, and it favored biventricular repair in 20% of patients who had the Norwood procedure. CONCLUSIONS: Morphologic and functional factors can be used to predict optimal pathway and survival benefit in neonates with critical left ventricular outflow obstruction.

Aortic Valve Stenosis↗
PubMed1998

Malignant fibrous histocytoma of the heart presenting as right ventricular outflow tract obstruction: a case report.

Primary malignant fibrous histocytoma (MFH) of the heart is extremely rare. Herein, we report the case of a 17-year-old woman with a primary MFH in the right ventricular outflow tract, presenting with increasing dyspnea on exertion. Wide excision of the tumor, including part of the pulmonary artery and pulmonary valve, was performed under cardiopulmonary bypass. Her postoperative recovery was uneventful.

Adolescent↗
PubMed2002

Critical pulmonary stenosis with intact ventricular septum and fetal arrhythmias.

A 23-year-old woman, gravida 1, was referred to our hospital for possible fetal distress at 32 weeks of pregnancy. A fetal cardiotochogram showed a reactive pattern, but mild continuous bradycardia and an intermittent pulse were observed, regarded as a sinus type and a type of A-V block, respectively. The continuous deceleration of the heart rate to 95 bpm was observed frequently at 35 weeks, 3 days of gestation. Thus, an emergent cesarean section was performed and a viable 2,082 g female infant was delivered. The neonate gradually became cyanotic, and an echocardiogram was performed. The neonate was regarded as a right ventricular outflow obstruction with intact ventricular septum. Unlike other cases, the infant revealed a moderately developed right ventricle despite a severely stenotic tricuspid valve. The infant died 27 days after birth and an autopsy established the diagnosis of critical pulmonary stenosis with intact ventricular septum. Right ventricular myocardial sinusoidal-coronary artery connections, one of the major features of this type of heart anomaly, was speculated to be involved in the cause of fetal bradyarrhythmias.

Adult↗
PubMed1999

Echocardiographic findings of pulmonary atresia or critical pulmonary stenosis and intact ventricular septum in utero.

BACKGROUND: Neonates with right ventricular outflow obstruction and intact ventricular septum show serious hemodynamic problems, such as severe hypoxemia, congestive heart failure due to massive tricuspid regurgitation, respiratory distress related to huge pulmonary hypoplasia or ventricular dysfunction due to right ventricle-coronary communication. Recent advances in fetal diagnosis include many cases of in utero diagnosis of pulmonary atresia or critical pulmonary stenosis and intact ventricular septum. METHOD: Among the fetuses examined from April 1994 to March 1998, five fetuses were found with pulmonary atresia (PA) or critical pulmonary stenosis (CPS). Fetal echocardiograms were reviewed to elucidate the accuracy of fetal information and the efficacy of fetal diagnosis in the perinatal management of patient with CPS or PA and intact ventricular septum. RESULTS: The five cases were divided into two groups: two with a very small right ventricle (group 1) and three with a tripartite right ventricle (group 2). Fetal cardiomegaly and right atrial dilatation were prominent in group 2, whereas cardiac sizes were normal in group 1. Serial fetal examination in one group 2 fetus revealed developing right ventricular hypertrophy in utero. All group 2 cases showed massive tricuspid regurgitation (TR). Estimated right ventricular pressures from TR always exceeded the systemic blood pressures of gestational age-matched neonates. Reversed flow through the ductus arteriosus was recorded in both groups and ductus-dependent pulmonary circulation after birth was anticipated. Patency of both tricuspid and pulmonary valves was difficult to recognize in utero, as was right ventricle-coronary artery communication. Four of the five cases were maternally transported and survived palliative and/or definitive intervention. One fetus with chromosomal abnormality was observed without intervention during infancy and received palliative surgery when she was two years old. CONCLUSION: Fetal hemodynamic information was useful for making decisions not only after birth, but also in utero, and may eventually result in improving the prognosis of babies with PA/CPS. Serial observation of the fetuses with PA/CPS may also suggest the possible pathogenesis of PA/CPS in utero.

Echocardiography↗
PubMed1988

Prediction of severe obstruction to right ventricular outflow after repair of tetralogy of Fallot and pulmonary atresia.

Cineangiograms were available for a quantitative retrospective study along with complete clinical information in 96 patients who underwent intracardiac repair of tetralogy of Fallot with pulmonary atresia. Multivariate analysis determined that the risk factors for too high a ratio (greater than or equal to 1) between the peak pressure in the right ventricle and that in the left, in the operating room about 30 minutes after repair, were as follows: size of the patient, small size of the right and left pulmonary arteries, and a larger number of large aortopulmonary collateral arteries. When, according to the multivariate equation, the predicted probability of this ratio being equal to or greater than 1 is 50% or more, consideration may be given to preliminary operations before repair; when the predicted probability is 70% or more, complete repair at that stage may be unwise.

Body Surface Area↗
PubMed1986

[Metastatic thyroid cancer to the right ventricle causing obstruction of the right ventricular outflow tract and associated with disseminated intravascular coagulopathy: a case report].

A case of thyroid cancer causing right ventricular outflow tract obstruction is described. A 72-year-old woman was admitted because of shortness of breath, some ecchymoses, and marked anasarca. Her liver was palpable four fingerbreadths below her costal margin. Laboratory findings included leukocytosis, marked thrombocytopenia, and an increase in fibrinogen degradation products due to disseminated intravascular coagulopathy. Two-dimensional echocardiography demonstrated a solid mass in the right ventricle, which protruded into the right atrium and main pulmonary artery. Right ventricular outflow tract obstruction and tricuspid regurgitation were demonstrated by contrast echocardiography. These findings were confirmed by CT scans, RI angiography, and contrast angiography. The mass was partially resected from her right ventricle and her tricuspid valve was replaced successfully, but she died of sepsis three weeks after surgery. At autopsy, undifferentiated thyroid cancer and cardiac metastasis were verified. To date, only eight cases with initial symptoms of congestive heart failure due to right ventricular outflow tract obstruction caused by metastatic intracavitary tumors have been reported. Very rarely have cardiac tumors resulted in disseminated intravascular coagulopathy.

Aged↗
PubMed1991

Reoperation for persistent outflow obstruction in hypertrophic cardiomyopathy.

This study compares results of a second left ventricular myotomy and myectomy (M + M) with those of mitral valve replacement (MVR) as reoperative procedures for persistent left ventricular outflow obstruction after M + M in hypertrophic cardiomyopathy. Comparison of the second M + M group (n = 12) with the MVR group (n = 11) disclosed significant difference (p less than 0.05) in mean age at the initial operation (29 +/- 11 years versus 40 +/- 8 years), interval between operations (46 +/- 57 months versus 18 +/- 13 months), and age at reoperation (33 +/- 10 years versus 42 +/- 8 years); and insignificant differences in mean preoperative functional class, cardiac index, left ventricular outflow gradients at rest or with provocation, and hospital mortality at reoperation (2/12 versus 1/11). At 6 months after reoperation, comparison of results of a second M + M with MVR showed that mean functional class, cardiac index, and left ventricular outflow gradient at rest were similarly improved, but the outflow gradient with provocation was significantly higher in the second M + M group (57 +/- 44 mm Hg versus 14 +/- 9 mm Hg, p less than 0.05). Total follow-up was 108 patient-years (100% complete) with an average of 5.9 years per patient in the second M + M group and 3.4 years per patient in the MVR group. Actuarial survival, including hospital mortality, at 3 and 5 years was 83% and 76%, respectively, after the second M + M, which was similar to 92% and 77% after MVR.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMedDate not supplied

Distribution of symptomatic congenital heart disease in Hong Kong.

Racial group studies have identified differences in the occurrence of congenital heart disease (CHD) among ethnic populations. The aim of this study was to characterize the proportionate frequency and clinical profile of children with symptomatic cardiac abnormalities in Hong Kong. The hospital records of 666, mainly Southern Chinese children with symptomatic CHD, who were 4 years of age or younger and who were admitted to Grantham Hospital, Hong Kong, in 1994 and 1995 were analyzed retrospectively. Left-to-right shunting (45.0%) and pulmonary outflow obstruction (34.4%) were the most frequently diagnosed categories, followed by left ventricular outflow obstruction (8.3%), transposition of the great arteries (4.2%), conditions with intracardiac mixing (3.9%), and other cardiac lesions (4.2%). Compared with Western studies, pulmonary outflow obstruction (p<0.0001), particularly tetralogy of Fallot and critical pulmonary stenosis, were more frequent in Chinese children. In contrast with previous reports, coarctation of the aorta (5%) does not seem to be uncommon in Chinese patients. Conversely, aortic stenosis and hypoplastic left ventricle may be rare in these children (1% vs 3% and 3-7%). Other cardiac lesions showed no consistent racial difference in the frequency of occurrence. Chinese patients with Down's syndrome had ventricular septal defect (38%) as the predominant lesion followed by atrioventricular septal defect (25%). Western studies usually report a reverse pattern for these two lesions. The mortality rate for the total cohort was 7.5%. However, of those with conditions with intracardiac mixing and left ventricular outflow tract obstruction many did not survive childhood (20% and 21%, respectively).

Analysis of Variance↗
PubMed1982

Homograft replacement of aortic root with reimplantation of coronary arteries. Results after one to five years.

Between 1976 and 1980, 26 patients aged 7 to 36 years had complete replacement of the aortic root with a valved homograft into which the coronary arteries were reimplanted. The main indication was the tunnel type of obstruction combining a hypoplastic valve ring, often with supra and subvalvar stenosis. Nineteen had previous operations for congenital left ventricular outflow obstructions. There was one perioperative death and one late death from progressive pulmonary vascular disease. Relief of left ventricular outflow tract obstruction was achieved in a majority of cases and the valves were entirely competent. With increasing experience, the initial problems of malalignment and torsion of the coronary arteries and complete heart block have been largely overcome. This operation provides an alternative technique for the management of diffuse left ventricular outflow tract obstruction and related problems in young patients. The long-term results of aortic homografts are well documented, and by eliminating the problems of aortic regurgitation it is anticipated that this may represent an advance in treatment.

Adolescent↗