Case 6 / 2015 - A 27-Year-Old Male Patient with Double Aortic and Pulmonary Valve Lesion after Double Valvotomy in Childhood

Clinical data: The patient underwent correction of congenital pulmonary and aortic valve stenosis, both with manifestations, at 7 years of age. Afterwards, there was progressive residual lesion of both valves, with predominance of regurgitation, and development of acute arrhythmias such as paroxysmal atrial fibrillation, in addition to ventricular extrasystoles. The obstructions were significant, with pressure gradients of 90 and 60 mmHg in the pulmonary and aortic valves, respectively. The patient was asymptomatic, and the loud systolic murmur was accompanied by thrill all over the precordium and neck vessels. There was right ventricular overload on the electrocardiogram (ECG). Double valvoplasty in the three-leaflet valves resulted in a good anatomical solution initially. To date, the patient reports shortness of breath on moderate exertion and precordial palpitations. He is on antiarrhythmic drug (amiodarone), after atrial fibrillation was controlled. 
 
Physical examination: Good general state of health, normal breathing, acyanotic, normal pulses. Weight: 77 kg; height: 181 cm; blood pressure (BP): 110/70 mmHg; and heart rate (HR): 51 bpm. The aorta (Ao) was moderately palpable on the suprasternal notch. 
 
The apical impulse was not palpable on the precordium, and there were mild systolic impulses on the left sternal border. Heart sounds were normal, and there was a grade 1-2/4 coarse systolic murmur in the pulmonic and aortic areas, and a grade 1-2/4 coarse diastolic murmur along the left sternal border. The liver was not palpable and the lungs were clear to auscultation.

DOI: 10.5935/abc.20140214 Clinical data: The patient underwent correction of congenital pulmonary and aortic valve stenosis, both with manifestations, at 7 years of age. Afterwards, there was progressive residual lesion of both valves, with predominance of regurgitation, and development of acute arrhythmias such as paroxysmal atrial fibrillation, in addition to ventricular extrasystoles. The obstructions were significant, with pressure gradients of 90 and 60 mmHg in the pulmonary and aortic valves, respectively. The patient was asymptomatic, and the loud systolic murmur was accompanied by thrill all over the precordium and neck vessels. There was right ventricular overload on the electrocardiogram (ECG). Double valvoplasty in the three-leaflet valves resulted in a good anatomical solution initially. To date, the patient reports shortness of breath on moderate exertion and precordial palpitations. He is on antiarrhythmic drug (amiodarone), after atrial fibrillation was controlled.
The apical impulse was not palpable on the precordium, and there were mild systolic impulses on the left sternal border. Heart sounds were normal, and there was a grade 1-2/4 coarse systolic murmur in the pulmonic and aortic areas, and a grade 1-2/4 coarse diastolic murmur along the left sternal border. The liver was not palpable and the lungs were clear to auscultation.
Chest radiograph showed moderately enlarged cardiac silhouette due to enlarged atrial and ventricular arches; the pulmonary vascular network was normal. Cardiomegaly progressed since the surgical correction, and the current cardiothoracic ratio was 0.58 (Figure 1). Clinical reasoning: during the course of the disease, the clinical elements were consistent with the diagnosis of double pulmonary and aortic valve lesion, with predominance of the first. Shortness of breath and development of ventricular arrhythmias and paroxysmal atrial fibrillation are related to the residual lesions, which progressively increased since childhood. Noteworthy, despite the evident biventricular dilatation, no electrical overload of these chambers was observed. Perhaps both ventricular dilatations had electrically counterbalanced one another.

Echocardiogram
Differential diagnosis: Concomitant lesion of both semilunar valves as congenital defects is usually associated with some genetic syndrome, which was not the case. Residual lesions of both valves commonly occur after correction of obstructive defects alone or in association, as occurs in the tetralogy of Fallot.
Management: in view of the progression of the residual defects with excessive dilatation of both ventricles, although with biventricular function still preserved, a surgical approach was chosen with valve replacement for a mechanical prosthetic valve in the aortic position and for a biological prosthetic valve in the pulmonary position. Because of the ascending aorta dilatation, a dacron tube was inserted inside the vessel.