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PubMed1998

Echocardiographic hemodynamic and morphometric predictors of survival after two-ventricle repair in infants with critical aortic stenosis.

OBJECTIVES: The purpose of this study was to identify echocardiographic hemodynamic and morphometric factors that would predict which infants with critical aortic stenosis could undergo relief of left ventricular outflow obstruction as opposed to the Norwood procedure. BACKGROUND: Echocardiographic predictors of survival in infants with critical aortic stenosis after two-ventricle repair have been mainly limited to morphometric factors, which have limitations. Echocardiographic hemodynamic predictors of survival in these patients have not previously been studied. METHODS: Doppler color flow mapping and pulsed Doppler techniques were used to obtain hemodynamic measurements of flow in the ascending, transverse and descending aorta, the ductus arteriosus, and across the aortic and mitral valves in infants with critical aortic stenosis. Morphometric measurements of the left heart structures were obtained, and comparisons were made between survivors and nonsurvivors for the hemodynamic and morphometric factors. RESULTS: Twenty-eight infants (mean age 1 +/- .6 days, mean weight 3.6 +/- .6 kg) with critical aortic stenosis were evaluated. Nineteen had a two-ventricle repair initially attempted, and nine had a Norwood operation. Among the patients with a two-ventricle repair, the hemodynamic factors associated with survival after two-ventricle repair included predominant or total antegrade flow in the ascending (p < 0.01) and transverse aorta (p < 0.05). Aortic valve gradient, mitral valve inflow and direction of flow in the ductus arteriosus and descending aorta were unrelated to outcome. The morphometric factors associated with survival after two-ventricle repair included the indexed aortic annulus (p < 0.0002), aortic root (p < 0.003), ascending aorta (p < 0.008) and left ventricular long-axis length (p < 0.01). Left ventricular volume, mass, ejection fraction and mitral valve area were not related to outcome after two-ventricle repair. CONCLUSIONS: In infants with critical aortic stenosis, predominant or total antegrade flow in the ascending and transverse aorta was associated with survival after two-ventricle repair. Determination of a one- versus two-ventricle repair remains a complex issue in infants with critical aortic stenosis. In addition to established morphometric predictors, hemodynamic information on the direction of flow in the aorta may help to define candidates for the Norwood operation.

Aortic Valve Stenosis↗
PubMed1980

[Differential diagnosis of obstructions to left ventricular outflow (author's transl)].

A total of 127 patients with different forms of obstruction to left ventricular outflow are studied. There were eight cases with supravalvular aortic stenosis, 79 with valvular aortic stenosis, 25 with hypertrophic subaortic stenosis, and 15 with subvalvular aortic stenosis. Clinical findings and electrocardiographic, radiographic, and angiohemodynamic data corresponding to these groups are reported, as well as the differential characteristics for each category from a clinical, electrocardiographic and radiographic point of view. The presence of a typical face, asymmetrical carotid and brachial pulses, absence of aortic ejection click and little or no aortic button on the chest roentgenogram reveals a supravalvular aortic stenosis. Valvular aortic stenosis shows aortic ejection click, poststenotic dilation of the ascending aorta, electrocardiographic signs of left ventricular hypertrophy and associated aortic regurgitation. Hypertrophic subaortic stenosis is characterized by a typical arterial pulse, marked "a" wave in the jugular venous pulse, double apical impulse on palpation and appearance or modification of the systolic ejection sound with Valsalva's maneuver.

Adolescent↗
PubMed1992

Development and determinants of dynamic obstruction to left ventricular outflow in young patients with hypertrophic cardiomyopathy.

BACKGROUND: To study the development of dynamic subaortic obstruction in young patients with hypertrophic cardiomyopathy (HCM), serial echocardiograms were retrospectively analyzed in a group of 26 consecutive children with this disease who showed no evidence of dynamic outflow obstruction at their initial evaluation (age, 11 +/- 3 years). METHODS AND RESULTS: After a follow-up of 3-12 years (mean, 7 +/- 3 years), seven of the 26 patients (27%) developed echocardiographic evidence of subaortic obstruction, i.e., marked systolic anterior motion (SAM) of the mitral valve with mitral-septal apposition and increased left ventricular outflow tract systolic velocities (3.8 +/- 0.3 m/sec; range, 3.1-4.5). Patients who developed SAM had smaller transverse dimension of the left ventricular outflow tract and more anteriorly displaced mitral valve when initially evaluated than did patients without development of SAM (outflow tract dimension, 19.1 +/- 4 versus 24.6 +/- 4 mm; mitral valve position index, 1.07 +/- 0.2 versus 0.73 +/- 0.3; each p less than 0.02). In patients with development of SAM, the already reduced outflow tract dimension decreased further during follow-up, and the mitral valve became even more anteriorly displaced within the left ventricular cavity. These developmental alterations in outflow tract size were associated with increases in left ventricular wall thickness, particularly of the basal anterior septum (11.0 +/- 8 mm; 72 +/- 33%) compared with control patients with HCM who did not develop SAM (3.0 +/- 3 mm; 17 +/- 10%; p less than 0.05). CONCLUSIONS: Development of subaortic obstruction in young patients with HCM results from a process of dynamic remodeling of left ventricular geometry over several years and is characterized by progressive narrowing of the outflow tract with anterior displacement of the mitral valve and disproportionate thickening of the basal anterior ventricular septum.

Cardiomyopathy, Hypertrophic↗
PubMed1992

Impact of dual-chamber permanent pacing in patients with obstructive hypertrophic cardiomyopathy with symptoms refractory to verapamil and beta-adrenergic blocker therapy.

BACKGROUND: Patients with obstructive hypertrophic cardiomyopathy (HCM) with symptoms refractory to drugs (beta-blockers or verapamil) are candidates for cardiac surgery (left ventricular septal myectomy or mitral valve replacement). The present study examines prospectively the ability of dual-chamber (DDD) pacing to improve symptoms and relieve left ventricular outflow obstruction in such patients. METHODS AND RESULTS: Forty-four consecutive patients with obstructive HCM who had failed to benefit from pharmacotherapy underwent treadmill exercise tests, echocardiography, and cardiac catheterization before and 1.5-3 months after implantation of a DDD pacemaker. Symptoms (angina, dyspnea, palpitations, presyncope, and syncope), New York Heart Association functional class status (1.7 +/- 0.7 versus 3.4 +/- 0.5, p less than 0.00001), and exercise durations were improved at follow-up evaluation. This was associated with significant reduction in left ventricular outflow tract gradient (38 +/- 38 versus 87 +/- 43 mm Hg, p less than 0.0001) and significant increases in cardiac output and systemic arterial pressures. Notably, when pacing was discontinued and comparisons were made in sinus rhythm, treadmill exercise durations were greater and left ventricular outflow tract gradients were less at the follow-up evaluation compared with the baseline study. CONCLUSIONS: DDD pacing is an effective alternative to surgery in most patients with obstructive HCM with drug-refractory symptoms. The beneficial effects of pacing continue to be evident when pacing is acutely discontinued.

Adrenergic beta-Antagonists↗
PubMed2006

Obstruction of the left ventricular outflow tract due to pannus formation after the implantation of a carbomedics aortic valve.

Excessive pannus formation after implantation of a prosthetic valve is an infrequent but serious complication. A 69-year-old woman who had received a 19-mm CarboMedics aortic valve 11 years ago was readmitted to our hospital with dyspnea and chest oppression. Cineradiography did not show the restriction of valve movement. The aortic peak pressure gradient was calculated by Doppler echocardiography to be 104 mmHg. Based on the diagnosis of stenosis of the left ventricular outflow tract, the patient underwent reoperation. At reoperation, the pannus had formed circumferentially without disturbing the movement of the leaflet. A 19-mm St. Jude Medical Regent valve was implanted after enlargement of the aortic annulus. The patient's postoperative course was uneventful. We report this characteristic finding of pannus formation after the implantation of a CarboMedics valve in the aortic position.

Aged↗
PubMed1994

Coronary flow dynamics and reserve assessed by transesophageal echocardiography in obstructive hypertrophic cardiomyopathy.

Myocardial ischemia is frequently associated with left ventricular outflow obstruction. To assess coronary flow impairment in obstructive hypertrophic cardiomyopathy (HC), 10 patients with echo-Doppler-detected obstructive HC and normal coronary arteries underwent transesophageal echo-Doppler examination of both coronary flow velocity (CFV) at rest, recorded in the proximal left anterior descending coronary artery, and coronary flow reserve (CFR) evaluated by means of dipyridamole infusion response. Ten normal patients were similarly studied and served as a control group. Two relevant alterations in coronary flow dynamics were detected in patients with HC: (1) a significantly increased diastolic/systolic CFV ratio, and (2) a significantly reduced dipyridamole/baseline CFV ratio. Compared with normal subjects, the CFV pattern showed a significantly greater diastolic and a significantly lower systolic component at rest (in some patients it was reversed). Diastolic/systolic CFV ratio was significantly higher in patients with HC at baseline (3.1 +/- 1 vs 1.6 +/- 0.5; p < 0.01) and increased further after dipyridamole infusion (4.9 +/- 2 vs 2.2 +/- 0.7; p < 0.01). In addition, CFR was impaired in patients with HC (1.8 +/- 0.3 vs 3.1 +/- 0.5; p < 0.01). Furthermore, a significant correlation between CFR and intraventricular pressure gradient was found. Thus, transesophageal echo-Doppler examination is a useful tool for evaluating CFV dynamics and CFR as demonstrated in patients with obstructive HC.

Aged↗
PubMed2006

Primary cardiac rhabdomyosarcoma causing obstruction to the right ventricular outflow.

A 72-year-old man presented with worsening shortness of breath and a systolic murmur. Transthoracic and transoesophageal echocardiograms revealed a huge mass (70 x 30 mm) obliterating the right ventricular outflow tract, with a peak transpulmonary gradient of 128 mmHg. Pathological examination confirmed the mass to be a primary cardiac rhabdomyosarcoma originating from the pulmonary valve.

Aged↗
PubMed1991

Intra-operative transoesophageal echocardiography after mitral repair--specific conditions and pitfalls.

The aim of this study was to determine the specific conditions and pitfalls of immediate assessment of the reconstructed mitral valve in open chest patients. Sixty-two patients had a mitral reconstruction controlled by transoesophageal echocardiography and colour Doppler (TEE) from the moment when cardiac activity restarted under extracorporeal circulation (ECC), until complete rewarming of the patient and weaning off the by-pass. The following conditions altered the imaging quality: (1) dried probe in the oesophagus or air bubbles, (2) non-aspirated air from the stomach, (3) small or empty left atria, (4) invagination of the left atrial appendage, (5) trapped air in the posterior pericardium, (6) spontaneous contrast. Transient mitral regurgitation disappeared completely in 12/62 patients: five had temporary impairment of LV function, two had reduced filling of the heart, one had left ventricular outflow obstruction exacerbated by isoproterenol and nitroglycerin, two had ventricular ectopic rhythms, and two epicardial pacing. Only one of the 62 patients had persistent significant MR which required a second run of ECC. We conclude that mitral regurgitation after mitral valve repair is closely related to the quality of LV function. A decision to reoperate should eliminate the possible pitfalls, and take into consideration the specific conditions of immediate postoperative cardiac function.

Echocardiography, Doppler↗
PubMed1986

Mitral valve stenosis and pure tricuspid valve regurgitation: comparison of necropsy patients having simultaneous mitral and tricuspid valve replacements with necropsy patients having simultaneous mitral valve replacement and tricuspid valve anuloplasty.

Clinical and morphologic observations are described in 30 patients (23 [77%] of whom were in functional class III or IV) who underwent replacement of the mitral valve for mitral stenosis and either simultaneous replacement (13 patients, group I) or anuloplasty (17 patients, group II) of the tricuspid valve for pure tricuspid valve regurgitation. Comparison of the 13 patients in group I with the 17 patients in group II disclosed similar mean ages (55 vs 58 years), similar average preoperative right ventricular systolic pressures (64 vs 61 mm Hg), similar average right atrial mean pressures (10 vs 9 mm Hg), similar average left ventricular systolic pressures (126 vs 120 mm Hg), similar average pulmonary artery wedge-left ventricular mean diastolic pressures (16 vs 18 mm Hg), similar cardiac indexes (2.1 vs 2.0 liters/min/m2), similar mean heart weights (507 vs 535 g), and similar percents with grossly visible foci of left ventricular necrosis (15% vs 12%) and fibrosis (23% vs 12%). Of the 13 patients in group I, 10 (77%) died early (less than or equal to 60 days of tricuspid valve replacement) and 3 (23%) died late (29, 37 and 120 months); of the 17 patients in group II, 14 (82%) died early and 3 (18%) died late (4, 9 and 98 months). The causes of early death in the 2 groups were different: of the 10 patients in group I who died early, the cause was excessive bleeding in 5, low cardiac output of undetermined etiology in 3, dysfunction of both prostheses in 1 and cerebral insult in 1; of the 14 patients in group II who died early, none died from excessive bleeding, 4 from decreased cardiac output of uncertain cause, 5 from left ventricular inflow obstruction (produced by a Starr-Edwards ball-valve prosthesis in 4 and from a Starr-Edwards disc prosthesis in 1), 1 from left ventricular outflow obstruction (by a porcine bioprosthesis), 2 from technical mishaps (incision into left ventricular free wall with rupture in 1 and ligation of the left circumflex coronary artery with resulting acute myocardial infarction in 1) and 2 died suddenly for reasons not determined. Of the 6 patients dying greater than 60 days after operation, 4 died from chronic congestive cardiac failure, 1 from a cerebral embolus and 1 from prosthetic valve endocarditis.

Adult↗
PubMed1995

[Dynamic obstruction of the left ventricular outflow tract by prosthesis dysfunction].

We report on a case of dynamic left ventricular (LV) outflow tract obstruction combined with a prosthetic valve dysfunction 13 years following mitral valve replacement with a Hancock bioprosthesis in a 46-year-old patient. Previously, repeated echocardiographic controls had been performed at regular intervals and the prosthesis had been found to be oversized and seated in abnormal position, with projection of the struts into the LV outflow tract. Moreover, a mild chronic LV outflow tract obstruction had been diagnosed upon intermittent findings of relatively high velocity in the outflow tract. Clinically, however, the patient had been stable and the function of the prosthesis had been good. With the onset of the mitral regurgitation due to prosthesis failure, a dynamic obstruction to LV outflow occurred, with severe narrowing of the LV outflow tract by a strut of the bioprosthesis during systole. Subsequently, a low cardiac output syndrome developed. The patient was referred for a mitral valve reoperation. The valve was replaced with a Sorin Bileaflet Carbon prosthesis. On the setting of a mild chronic LV outflow obstruction due to the oversized prosthesis and its abnormal position, hypercontractile cardiac function as a result of mitral regurgitation may have caused the dynamic and symptomatic LV outflow tract obstruction.

Bioprosthesis↗
PubMed1977

Supracristal ventricular septal defects: spectrum of associated lesions and complications.

The clinical records and radiographs of 46 patients with a supracristal ventricular septal defect are reviewed to illustrate the spectrum of associated lesions and complications seen with this defect. The presence of a supracristal ventricular septal defect compromises the normal conal muscular support of the aortic valve, usually in the region of the right sinus of Valsalva, and, consequently, complications involving the aortic valve are frequently noted. Decreased sinus support may lead progressively to prolapse of the right aortic cusp through the supracristal ventricular septal defect into the right ventricular infundibulum effecting sequentially aortic regurgitation, right ventricular outflow obstruction, and ultimately right sinus of Valsalva-right ventricular fistula. This potential progression is an indication for early recognition and repair of the supracristal ventricular septal defect. A supracristal ventricular septal defect is also an integral component of the Taussig-Bing malformation, is present in the majority of patients with aortic arch interruption, and may be seen in as many as 25% of Oriental patients with tetralogy of Fallot.

Adolescent↗
PubMed1996

[Right ventricular myxoma. A rare case of pulmonary stenosis].

We discuss a case of a fourteen year old girl in whom, clinical signs of right ventricular outflow obstruction were discovered following a syncopal attack. A right ventricular tumor was observed by echocardiography. Histology confirmed the diagnosis of myxoma.

Adolescent↗
PubMed2003

Cardiac metastasis from a renal cell carcinoma.

Renal cell carcinomas are known to extend into the renal vein and inferior vena cava, but cardiac metastases are rare and usually clinically silent. In the case described here, there was evidence of right ventricular outflow obstruction, associated with a metastatic renal tumour in the right ventricular wall protruding into the ventricular cavity. The patient had presented years earlier with an ulnar neuropathy, for which the tumour may have been responsible. This case highlights the need to consider an underlying paraneoplastic syndrome in a patient presenting with neuropathy, because appropriate investigation could have led to early detection and possible cure of the renal lesion. When renal cell carcinoma is confined to the renal parenchyma, five-year survival is up to 70%, but this falls to 5% in the presence of distant metastases.

Aged↗
PubMed1980

Frequency and direction of interatrial shunting in valvular pulmonic stenosis with intact ventricular septum and without left ventricular inflow or outflow obstruction. An analysis of 127 patients treated by valvulotomy.

This report summarizes observations in 127 patients who underwent pulmonic valvulotomy for valvular pulmonic stenosis with intact ventricular septum and without obstruction to left ventricular inflow or outflow. Of the 127 patients, 30 (24%) preoperatively by dye dilution curves had shunting at the atrial level: in 19 (63%), the shunt was right-to-left, and in the other 11 (27%), entirely left-to-right. The patients with right-to-left interatrial shunts had severe pulmonic valve stenosis (average peak systolic pressure gradient = 120 +/- 11 mm. Hg) and small (average diameter 1.1 +/- 0.1 cm.) sized defects in the atrial septum (patent foramen ovale). In contrast, the patients with left-to-right shunts had mild to moderate pulmonic valve stenosis (average peak systolic pressure gradient = 60 +/- 5 mm. Hg) and relatively large (average diameter = 2.8 +/- 0.1 cm.) defects in the atrial septum (true atrial septal defect). The patients with right-to-left interatrial shunts had no significant differences in right versus left atrial pressures. The patients with left-to-right interatrial shunts, however, had left atrial pressures significantly greater than right atrial pressures (7 +/- 0.5 vs 5 +/- 0.5, p less than 0.05). No significant differences were found in ventricular end-diastolic pressures.

Adolescent↗
PubMed1977

USE OF AN EXTRACARDIAC CONDUIT IN THE REPAIR OF l-TRANSPOSITION WITH l-LOOPING ASSOCIATED WITH SEVERE SUBVALVULAR PULMONIC STENOSIS AND A VENTRICULAR SEPTAL DEFECT.

Total correction was performed in a child with l-transposition of the great arteries, severe subpulmonic stenosis, and a ventricular septal defect. The subpulmonic obstruction was bypassed with an extra-cardiac valved conduit. This alternate method of relieving ventricular outflow obstruction should be considered when conventional techniques cannot be employed because of complex intracardiac anatomy. Proper placement of the pulmonary ventriculotomy is important in order to avoid injury to coronary arteries on the upper and lower ventricular wall and to papillary muscles in the mid-portion of the ventricle.

Journal Article↗