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1/6. Low pressure giant pulmonary artery aneurysms in the adult: natural history and management strategies.

    OBJECTIVE: To describe aspects of the natural history and pathophysiology of giant low pressure pulmonary artery aneurysms and to propose potential surgical strategies. DESIGN: Cross sectional retrospective review. SETTING: Supraregional tertiary referral centre. patients: All adult patients referred for assessment of giant pulmonary artery aneurysm retrospectively identified from the Mayo adult Congenital heart disease Clinic database. methods: Patient data were reviewed from hospital records, including echocardiograms, magnetic resonance images, radiographs, and histology slides. RESULTS: Four patients were identified with a median age of 52 years (range 37-64 years). Presenting symptoms were effort related dyspnoea, chest discomfort, and hoarseness in one patient. All patients had pulmonary regurgitation and clinical evidence of right ventricular enlargement in association with a pulsatile mass at the upper left sternal edge. Transthoracic echocardiography showed the giant pulmonary artery aneurysm involving the main pulmonary artery and proximal branches, and confirmed severe pulmonary regurgitation in all patients. None of the patients had intimal tearing, medial dissection, or pulmonary arterial rupture. The pulmonary valve was replaced to relieve symptoms and preserve right ventricular function. Pulmonary arterial histology showed medial degeneration of elastic fibres with accumulation of basophilic ground substance. CONCLUSIONS: rupture or dissection of these low pressure aneurysms is rare. The timing of surgical intervention should be determined by changes in right ventricular size and function resulting from pulmonary regurgitation or pulmonary stenosis, and not the size of the aneurysm.
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2/6. Anasarca due to pulmonic valve regurgitation with low pulmonary vascular resistance.

    A 57-year-old woman underwent pulmonic valvotomy for congenital pulmonic stenosis. She developed severe pulmonic insufficiency, secondary tricuspid regurgitation, and anasarca in spite of a normal pulmonary artery pressure. Insertion of a pulmonary valve prosthesis and tricuspid valve plication reversed all clinical symptoms and signs of this rare complication of pulmonary valvotomy.
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3/6. Repair of pulmonary valve insufficiency using an autologous monocusp.

    A 3 1/2-year-old boy experienced right ventricular failure approximately two years after an operation for critical pulmonic stenosis. Severe pulmonary and tricuspid valvular insufficiency was documented echocardiographically and at cardiac catheterization. Treatment consisted of a tricuspid valve annuloplasty and creation of an autologous monocusp valve using the anterior wall of the pulmonary artery. The procedure was well tolerated, and early competence of the pulmonary valve was shown intraoperatively by pressure recordings and postoperatively by Doppler echocardiography. However, at recatheterization one year later, there was no evidence that the monocusp valve was functioning. This experience does not support the prior successful application of this technique in animals.
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4/6. Noninvasive evaluation of intracardiac pressures using Doppler ultrasound: a case study of panvalvular regurgitation.

    Doppler velocity signals from regurgitant valve flow can be used to calculate pressure gradients across incompetent valves by a modification of the Bernoulli equation. Analysis of these gradients provides clinically useful, noninvasive information about cardiac chamber pressures. This study presents an unusual case of cardiomyopathy with panvalvular regurgitation which demonstrates the major methods of pressure analysis using Doppler signals from regurgitant valves.
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5/6. Unusual clinical and echocardiographic features of severe isolated pulmonic insufficiency.

    A 57-year-old psoriatic man had severe, isolated pulmonic regurgitation, with intractable right sided failure. echocardiography showed flail pulmonic leaflet and premature opening of the valve. cardiac catheterization documented severe pulmonic regurgitation. The right ventricular end-diastolic pressure was elevated above the pulmonary artery diastolic pressure, thus explaining the echocardiographic finding. The patient was treated successfully by pulmonic valve replacement.
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6/6. Early pulmonary homograft failure from dilatation due to distal pulmonary artery stenosis.

    Early progressive pulmonary homograft insufficiency developed in an 11-month-old infant after repair of truncus arteriosus because of dilatation secondary to the presence of residual distal pulmonary artery stenosis and hypoplasia. Before repair, the pulmonary artery branches were discontinuous, with the right pulmonary artery being somewhat hypoplastic and originating from the trunk, and the left pulmonary artery supplied by a modified Blalock-Taussig shunt created in the newborn period. At repair, a pulmonary homograft was used to connect the branches. Progressive cardiomegaly and oxygen dependance occurred 3 weeks postoperatively. cardiac catheterization showed systemic right ventricular pressure, severe homograft insufficiency, and residual distal pulmonary artery stenosis and hypoplasia. On reoperation at 3 months postoperatively, the homograft annulus diameter increased from 14 mm to 16 mm. dilatation and insufficiency probably occurred because the right ventricle and homograft distal to the obstruction functioned as a unit during systole. The problem might have been minimized with the use of aortic homograft, which is thicker, or annular reinforcement with a synthetic material.
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