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Left ventricular outflow obstruction after mitral valve repair (Carpentier's technique). Proposed mechanisms of disease.

Left ventricular outflow tract obstruction (LVOTO) after mitral valve repair by Carpentier's technique has been recently reported in the literature. To assess the mechanisms of this phenomenon, we investigated 307 mitral valve repairs performed between July 1985 and December 1986. Incidence of LVOTO related to the mechanism of the mitral insufficiency and to the etiology demonstrates a direct relation to preoperative mitral valve prolapse (posterior leaflet +/- anterior leaflet) of degenerative origin. No LVOTO occurred after rheumatic mitral insufficiency repair regardless of size of the left heart cavities or of the prosthetic ring. Intraoperative and surficial two-dimensional echocardiography, color Doppler methods, and cardiac catheterization were used to investigate the mechanisms leading to LVOTO. Nonspecific modifications induced by reduction in size of the mitral annulus by the prosthetic ring (anterior displacement of the posterior ventricular wall and of the posterior mitral leaflet and narrowing of the mitroaortic angle) are not sufficient to explain the LVOTO. The association of mitral leaflets (composed of excess tissue and opposed to flow by a perpendicular position attributable to a narrow mitroaortic angle) and geometric left ventricular modifications (responsible for the superposition of mitral inflow to ventricular outflow) also qualifies as a mechanism for the induction of LVOTO after mitral surgical repair.

Cardiac Catheterization↗

Management of univentricular heart with systemic ventricular outflow obstruction by pulmonary artery banding and Damus-Kaye-Stansel operation.

BACKGROUND: Some patients with univentricular hearts who are candidates for Fontan operation may develop ventricular outflow tract obstruction after pulmonary artery banding (PAB) or Fontan. However, the indication for Damus-Kaye-Stansel (DKS) operation for these patients has not been clear. To clarify the indication, the changes in the diameter of ventricular outflow tract and the feasibility of DKS operation before or with Fontan were investigated. METHODS: Among the patients with univentricular heart who underwent PAB, 21 patients had probable ventricular outflow obstruction with an aorta arising from the morphologic right ventricle. Diameter of ventricular outflow tract was measured before and after PAB, Glenn, and Fontan operations with or without DKS, and indexed by normal value (%VOT). RESULTS: Six patients died after PAB. In the surviving 15 patients, %VOT decreased significantly from 103% (median, range 75%-153%) to 75% (range 52%-153%) after PAB. Four with very small %VOT (52% to 63%) after PAB needed DKS with bidirectional Glenn or central shunt operation, and 5 with moderately small %VOT (67% to 109%) after PAB needed DKS concomitantly with Fontan. A patient with %VOT of 117% before Fontan required DKS after Fontan. A patient with %VOT of 153% underwent Fontan without DKS and obstruction did not develop after Fontan. The remaining 4 patients were under consideration for Glenn or Fontan operation. CONCLUSIONS: The diameter of the ventricular outflow tract decreased after PAB and Fontan operations. DKS operations might be indicated before Fontan if the indexed diameter of ventricular outflow tract after PAB was below 70% and concomitantly with Fontan if it was below 120%.

Cardiac Surgical Procedures↗

Left ventricular outflow obstruction in Taussig-Bing malformation.

In a patient recently undergoing total intracardiac repair of the Taussing-Bing malformation the immediate cause of death appeared to be an obstruction to left ventricular outflow. Examination of this and other specimens of Taussig-Bing malformations revealed that the obstruction appeared to be a portion of the pulmonary conus. This area may be overlooked in the preoperative evaluation and is so located that the obstructive nature of this muscle mass may not be appreciated at the time of surgical repair. The obstructive part of the pulmonary conus can be excised and this appears to be a more common component of the Taussig-Bing anomaly than has previously been appreciated.

Angiocardiography↗

Left ventricular outflow obstruction after aortic valve replacement: detection with continuous wave Doppler ultrasound recording.

A 33 year old woman developed left ventricular outflow obstruction due to a fibrous subvalvular membrane 2 years after insertion of a Hancock modified orifice, porcine aortic valve prosthesis. M-mode and two-dimensional echocardiographic studies failed to detect the membrane. Continuous wave Doppler study revealed a maximal velocity of 4.8 m/s and a calculated peak pressure gradient of 92 mm Hg between the left ventricle and aorta. This finding was in close agreement with the peak gradient of 100 mm Hg obtained at cardiac catheterization. Because of its ability to accurately measure valvular gradients, continuous wave Doppler ultrasound study appears to be a useful noninvasive technique in the evaluation of patients with suspected prosthetic valve dysfunction.

Adult↗

Left ventricular outflow obstruction in rhabdomyoma of infancy: meta-analysis of the literature.

Primary heart tumors are exceptional in infants and children. Most common is the rhabdomyoma, often associated with tuberous sclerosis (Bourneville's disease). This tumor is generally believed to have no hemodynamic effects in the majority of cases. Recently, severe obstruction of the left ventricular outflow tract by a solitary tumor was diagnosed during pregnancy and emergency surgery was needed soon after birth.

Female↗

[Right ventricular outflow obstruction due to a giant pseudoaneurysm of the anterior descending coronary artery in a patient with Behçet's disease].

Giant pseudoaneurysms of coronary arteries in patients with Behçet's disease is a uncommon finding. It has been described exceptionally in the literature. We present a case of giant pseudoaneurysm of the left anterior descending coronary artery with obstruction of the right ventricular outflow in a patient with Behçet's disease. He improved after surgical resection and steroid treatment.

Adult↗

Altered cardiac hemodynamic and electrical state in normal sinus rhythm after chronic dual-chamber pacing for relief of left ventricular outflow obstruction in hypertrophic cardiomyopathy.

Dual-chamber (DDD) pacing relieves left ventricular (LV) outflow tract obstruction in patients with hypertrophic cardiomyopathy. The reduction in LV outflow gradient persists in some patients after cessation of pacing. Twelve-lead and signal-averaged electrocardiograms were obtained before and after 12 weeks of DDD pacing in 18 patients with obstructive hypertrophic cardiomyopathy to determine whether the altered hemodynamic state after chronic pacing is accompanied by electrical changes. Hemodynamic studies were performed at baseline and at follow-up. Signal-averaged electro-cardiograms were obtained using a Corazonix Predictor and bidirectional filters at 25 Hz to a noise level of less than 0.5 microV. At follow-up, LV outflow tract gradients were reduced significantly during DDD pacing and with cessation of pacing in sinus rhythm by 56 +/- 10 and 47 +/- 10 mm Hg, respectively (p less than 0.001). There was no simple relation between changes in LV outflow tract gradient and in the electrocardiogram. For example, amplitude of the R wave in V5,6 was reduced by greater than or equal to 0.5 mv in 4 patients, unchanged in 12 and increased in 2. Similarly, the S wave in leads V1,2 was reduced in 7 patients, unchanged in 7 and increased in 4. The T wave became more negative (greater than or equal to 0.1 mv) in leads II, III, aVF and V5,6 in 13 patients and more positive in leads I and aVL in 12.(ABSTRACT TRUNCATED AT 250 WORDS)

Cardiac Pacing, Artificial↗

Early results of AV sequential pacing on left ventricular outflow obstruction after Senning procedure.

Dual chamber pacing was shown to decrease left ventricular outflow tract (LVOT) obstruction in patients with hypertrophic cardiomyopathy 30 years ago. We report early results of AV sequential pacing from the LV apex in a patient with transposition of the great arteries who is post-Senning procedure. LVOT obstruction resulted from septal deviation and systolic anterior motion of the mitral valve. Pacing was indicated for sinus node dysfunction. AV sequential pacing with a short optimal AV interval of 60 ms demonstrated a 45% reduction in the degree of LVOT obstruction. This article suggests that LVOT obstruction after the Senning procedure can be palliated by asynchronous septal contraction induced by AV sequential pacing, even if the activation is from LV apex, and avoid or postpone surgery in selected situations.

Adolescent↗

Dynamic Left Ventricular Outflow Obstruction in Hypertrophic Cardiomyopathy Revisited: Significance, Pathogenesis, and Treatment.

Systolic anterior motion of the mitral valve and mitral-septal contact is the usual cause of dynamic left ventricular outflow obstruction in hypertrophic cardiomyopathy. That true obstruction actually occurs is now established based on cardiac catheterization and echocardiographic evidence. A mid-systolic drop in left ventricular systolic ejection velocity because of obstruction has been demonstrated recently. Echocardiographic data indicate that systolic anterior motion of the mitral valve is initiated by flow drag; the mitral valve is swept toward the septum by the pushing force of flow. After mitral-septal contact, obstruction begets further obstruction as the pressure gradient pushes the mitral valve into the septum. Most symptomatic patients with obstruction can be treated successfully with negatively inotropic drugs. These medications reduce systolic anterior motion and obstruction by decreasing early left ventricular ejection acceleration, decreasing the early systolic pushing force on the protruding mitral leaflet. Patients who do not improve on medication generally benefit from surgery. Newer interventions to relieve obstruction, such as dual-chamber pacing and percutaneous transluminal septal myocardial ablation are under active investigation.

Journal Article↗

Coexistent mitral stenosis and dynamic left ventricular outflow obstruction.

A 66-year-old female with known mitral stenosis presented with symptoms of progressive pulmonary congestion. Concomitantly, subvalvular left ventricular outflow obstruction was suspected clinically and both lesions were confirmed at cardiac catheterization. At operation, marked septal hypertrophy and rheumatic mitral stenosis were observed, the mitral valve was replaced, and a septal myomectomy was performed.

Aged↗

[Left ventricular outflow obstruction due to accessory mitral tissue].

An asymptomatic 31-year-old woman was studied because she presented a systolic ejection cardiac murmur. The echocardiogram showed a mobile, sac-like mass attached to the ventricular surface of the anterior mitral leaflet and its chordae tendineae and papillary muscle. This structure moved to the left ventricular outflow tract during every systole occupying the subaortic area. Conventional and color-coded Doppler examination revealed left ventricular outflow obstruction caused by the accessory mitral valve tissue that produced a high-velocity turbulent flow pattern in the subaortic area where the sac-like structure approximated to the outflow tract walls.

Adult↗

Size of the normal aortic root in normal subjects and in those with left ventricular outflow obstruction.

This study establishes normal values and growth curves of the aortic root to determine when it is unusually small. The aortic root diameter was measured by 2-dimensional echocardiography in 188 normal subjects (group 1) and in 33 patients (group 2) with left ventricular outflow obstruction in whom the diagnosis of small aortic root was made during surgery for aortic root and valve replacement. In group 2 the aortic root was also measured by M-mode echocardiography and was compared to measurements during surgery. Growth curves of the normal aortic root were obtained. In group 2 the aortic root was smaller than normal except in 3 patients. When corrected for body surface area all were smaller than normal. The 2-dimensional echocardiographic and surgical measurements were almost identical. M-mode values were higher (p less than 0.01). The normal values and growth curves of the aortic root diameter are thus established and the small aortic root can be identified preoperatively.

Adolescent↗

Repair of common atrioventricular canal associated with transposition of the great arteries and left ventricular outflow obstruction.

A 9-year-old girl was successfully operated upon for transposition of the great arteries (TGA), complete atrioventricular (AV) canal, severe left ventricular outflow obstruction, absence of the coronary sinus, and a large left superior vena cava draining into the left upper corner of a common atrium. The interventricular communication was closed and the common AV orifice was partitioned, a Mustard operation was performed, and a valved conduit was inserted between the left ventricle and the pulmonary artery. This operation, which leads to a four-chamber heart, may be preferable for this condition to the previously reported Fontan type of repair. The same approach may be also used to correct those cases of double-outlet right ventricle with complete AV canal in which the interventricular communication does not extend into the perimembranous area.

Child↗

Experience with left ventricular apicoaortic conduits for complicated left ventricular outflow obstruction in children and young adults.

Six patients, ranging in age from 8 to 20 years, underwent left ventricular apicoaortic construction for treatment of complicated left ventricular outflow tract obstruction. All patients had severe left ventricular hypertrophy as determined by echocardiography and electrocardiography. The preoperative gradient across the left ventricular outflow tract was 84 +/- 17 mm Hg (mean +/- standard deviation) (range, 65 to 110 mm Hg), and the preoperative left ventricular end-diastolic pressure was 20 +/- 7 mm Hg (range, 12 to 28 mm Hg). Conduits were placed retroperitoneally with the distal anastomosis to the infrarenal aorta; the porcine valve was positioned in the left upper quadrant. Relief of left ventricular hypertension was complete; the minimal gradient measured intraoperatively was 13 +/- 8 mm Hg (range, 0 to 20 mm Hg). With an average follow-up of 18 months (range, 6 to 24 months), all patients have improved and are in Functional Class I. Four patients were catheterized 12 months postoperatively. They all showed excellent relief of left ventricular hypertension; the conduit was effectively decompressing the left ventricle. This experience suggests that this approach is effective in relieving complex left ventricular outflow tract obstruction with good early results, and that it deserves more frequent application in children.

Adolescent↗

Left ventricular outflow obstruction produced by a pedunculated fibroma in a newborn: clinical, angiographic, echocardiographic and surgical observations.

An unusual case of a one-day-old infant with significant left ventricular outflow obstruction produced by a pedunculated fibroma is described. The clinical findings were indistinguishable from severe valvular or subvalvular aortic stenosis. The tumor was difficult to detect by echocardiography. It produced an echo-free widening of the left ventricular outflow tract. Left ventricular cineangiography clearly demonstrated a mobile mass beneath the aortic valve.

Aortic Valve Stenosis↗

Relief of left ventricular outflow obstruction by cibenzoline in a patient with Fabry's disease--a case report.

A 46-year-old man was admitted for further evaluation of exertional chest discomfort. One family member had experienced sudden death, and 2 others had died of heart failure, including 1 known to have had Fabry's disease. The patient was also diagnosed with Fabry's disease, based on reduced leukocyte alpha-galactosidase A activity, 2.0 nmol/mg protein/hour, as well as endomyocardial biopsy findings of marked sarcoplasmic vacuolization of cardiac muscle cells by light microscopy and lamellated "zebra bodies'' in the cytoplasm shown by electron microscopy. Echocardiography disclosed marked left ventricular hypertrophy and systolic anterior motion of the mitral leaflets. On cardiac catheterization, a left ventricular peak systolic outflow gradient of 50 mm Hg was noted; this decreased to 10 mm Hg following intravenous administration of 100 mg of cibenzoline. It is imperative to recognize the existence of cases with Fabry's disease associated with left ventricular outflow obstruction.

Anti-Arrhythmia Agents↗

Right ventricular outflow obstruction secondary to post-infarction aneurysm: a possible new syndrome.

Two patients with extensive anterior myocardial infarction developed a hitherto unreported type of right ventricular outflow tract obstruction. A 71-year-old woman, who had had an acute infarction 10 years before, was admitted for sustained ventricular tachycardia. A loud ejection murmur was heard in the mid-precordium. The echocardiogram and left ventriculogram showed a septal aneurysm, with a systolic gradient of 21 mmHg between the right ventricular outflow tract and apex. The ejection murmur was detected in the outflow tract by intracardiac phonocardiography. The second patient was a 60-year-old man who had had an acute infarction at age 47. He was also referred because of ventricular tachycardia, and his clinical situation was almost the same as that of the first case. Our search of the literature failed to disclose any similar case with a loud ejection murmur confirmed by intracardiac phonocardiography to be due to an obstructive septal aneurysm.

Aged↗