[Clinical picture and hemodynamics of pulmonary stenosis without ventricular septal defect].
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Congenital aneurysm of the sinus of Valsalva is a rare cardiovascular anomaly. It is usually silent until rupture occurs. The natural history of unruptured aneurysm of the sinus of Valsalva is still not clear, and the therapeutic strategy is uncertain. Here we reported a case of unruptured aneurysm of the sinus of Valsalva which was correctly diagnosed before invasive diagnostic procedures. A 30-year-old female noted mild palpitation and dyspnea for 1 month. Physically, a grade 3/6 systolic ejection murmur at upper left sternal border was detected. Echocardiography revealed dilatation and irregular protrusion of the right sinus of Valsalva encroaching on right ventricular outflow tract to cause obstruction. With these findings, unruptured aneurysm of the sinus of Valsalva with pulmonary stenosis was diagnosed. Cardiac catheterization and angiography confirmed the diagnosis. The aneurysm was repaired with a Dacron patch with good results. It is concluded that sinus of Valsalva aneurysm can be diagnosed by echocardiography before its rupture so as to render a proper management for this potentially life-threatening anomaly.
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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.
OBJECTIVE: This study attempted to determine the optimal therapeutic interventions by risk-adjusted comparisons of early and intermediate-term outcomes. BACKGROUND: The variety of interventions and the small case load at individual institutions have delayed the generation of reliable information concerning therapy for this condition. METHODS: In this prospective 27-institution study, 101 neonates were consecutively enrolled (between January 1, 1987 and January 1, 1991). Treatment was determined by the physicians. Demographic and morphologic details were tabulated. Dimensions of the pulmonary "anulus" and tricuspid anulus were measured on echocardiograms, and right ventricular cavity size was estimated. Right ventricular-pulmonary trunk pressure gradients were tabulated. Numerous analyses were made. RESULTS: Severe pulmonary valve stenosis and an intact ventricular septum were present in all patients. The right ventricular-pulmonary trunk junction ("anulus") was severely narrowed in 15%. Right ventricular cavity size was severely reduced in 4%. The tricuspid valve was small in 15% of patients; its diameter was poorly correlated with right ventricular cavity size. Eighty-nine percent and 81% of patients survived > or = 1 month and 4 years, respectively, after the initial procedure. Multivariable analysis identified no patient-specific risk factors for death. Only open pulmonary valvotomy without a support technique was uniformly a procedural risk factor; under some circumstances, transannular patching without a shunt was a risk factor. The right ventricular-pulmonary trunk gradient immediately after valvotomy was < 30 mm Hg in 81% of patients and was similar after surgical and balloon valvotomy. In 74% of patients, no intervention was required after the first accomplished intervention. CONCLUSIONS: Marked variation in morphology is uncommon in critical pulmonary stenosis in neonates. Percutaneous balloon valvotomy and certain types of surgical valvotomy are optimal initial procedures. The unusual situation of a small pulmonary "anulus" may initially require a transannular patch and a systemic-pulmonary artery shunt.
We report a case of critical pulmonary valve stenosis in which congenital aneurysm of the membranous septum ruptured spontaneously after balloon dilatation of the pulmonary valve. It is considered that the chronic mechanical trauma with phasic protrusion and collapse of the aneurysm during the cardiac cycles was responsible for an aneurysm rupture.
BACKGROUND: Pulmonary regurgitation (PR) is an important determinant of outcome after repair of tetralogy of Fallot. Baseline PR was measured by magnetic resonance (MR) phase velocity mapping and from real-time right ventricular pressure-volume loops with a conductance catheter. Subsequently, the impact of two loading maneuvers (increased airway pressure, simulated branch pulmonary artery stenosis) on PR was assessed by the conductance catheter method. METHODS AND RESULTS: Thirteen patients, 3 to 35 years after tetralogy of Fallot repair or pulmonary valvotomy, had PR measured by MR phase velocity mapping while breathing spontaneously. During catheterization under general anesthesia. PR was estimated from right ventricular pressure-volume loops generated by conductance and microtip pressure catheters. The effect of increased airway pressure (continuous positive airway pressure, 20 cm H2O; n = 12) and simulated branch pulmonary artery stenosis (transient balloon occlusion of a branch pulmonary artery, n = 7) was measured. Basal PR fraction derived by MR and from right ventricular pressure-volume loops had a correlation coefficient of .76 and mean of differences of 2.0 +/- 18.2% (95% limits of agreement). Increased airway pressure increased PR (16.3 +/- 11.4% to 25.7 +/- 17.3%, P < .01). Simulated branch pulmonary artery stenosis increased right ventricular end-systolic pressure (69.1 +/- 21.4 to 78.7 +/- 23.1 mm Hg, P < .05) and PR (27.5 +/- 11.3% to 36.9 +/- 12.8%, P < .05). CONCLUSIONS: There was reasonable agreement between MR phase velocity-derived PR fraction and that obtained from right ventricular pressure-volume loops generated by use of conductance and pressure-microtip catheters. Exacerbation of PR by increased airway pressure and branch pulmonary stenosis may be relevant to the acute postoperative and long-term management, respectively, of patients after repair of tetralogy of Fallot.