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From 1969 to 1989, 15 patients with an aneurysm of the sinus of Valsalva underwent operative correction. This represents 0.23% of 6515 cardiac operations with cardiopulmonary bypass during that time. There were 8 males and 7 females ranging in age from 15 to 54 years (mean 35.8 years). Symptoms of congestive heart failure, fatigue and palpitation were common. All patients underwent cardiac catheterization including aortography. Associated lesions included aortic valve regurgitation in 6 patients and a ventricular septal defect in 3 patients. The following connections occurred: right coronary sinus to right ventricle (8 patients), right coronary sinus to both right atrium and right ventricle (1 patient), and noncoronary sinus to right atrium (6 patients). The aneurysm was repaired via aortotomy or through the chamber into which it emptied. The aortic valve was replaced in 2 patients. There were no early or late postoperative deaths. Fourteen patients were in NYHA functional class I at late follow-up (range 0.5 to 20.5 years, mean 8.7 years). There have been no recurrences. Our experience supports the concept that early surgical intervention in patients with ruptured aneurysms of the sinus of Valsalva is justified.
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PMID:Ruptured aneurysms of the sinus of Valsalva. 154 97

The most common congenital cardiac defect is VSD. This malady accounts for 20 to 30 per cent of all congenital cardiac defects and is representative of a cardiac lesion that increases pulmonary blood flow. Although lesions, which increase pulmonary blood flow, may vary in incidence, they frequently have common symptomatology. Over time, congestive heart failure becomes a problem. Poor respiratory status leads to weight loss, poor feeding, and failure to thrive. If unrepaired, the child often presents with cyanosis and tachypnea. The history may include frequent respiratory infections, exercise intolerance, generalized malaise, or fatigue. In spite of poor weight gain, the child may be edematous and have a large liver. Definitive diagnosis of each lesion may be made by echocardiogram, cardiac catheterization, or both. With these data and a detailed history, treatment and management decisions are determined. In most cases, as the child gets older, symptoms become more evident. This is the result of high pulmonary pressure. High pressure over time causes a thickening of the alveolar tissue, which decreases the permeability of the alveolar membranes for gas exchange. Lung changes can become irreversible, but it is unusual for irreversible changes to occur before 1 year of age. All the lesions described in this article are amendable to primary repair before 1 year of age, affording the best functional results. Postoperative nursing care includes management of persisting CHF and PVR while maintaining adequate cardiac output. Many factors, including electrolyte balance, nutritional status, conduction disturbances, stress, and parental anxiety, influence the management of these infants. The outcome depends greatly on the assessment skill of a highly competent cardiac intensive care nurse and an environment conducive to collaborative practice.
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PMID:Caring for patients with lesions increasing pulmonary blood flow. 281 77

Ruptured sinus Valsalva aneurysm was repaired in 13 patients (mean age c. 33 years). Dyspnea, chest pain, fatigue and palpitation were the most common symptoms and systodiastolic murmur, cardiomegaly and pulmonary congestion the most pertinent clinical findings. The pulmonary-to-systemic flow ratio averaged c. 2.5. Associated cardiac anomalies were ventricular septal defect, aortic or mitral regurgitation, aortic coarctation or subvalvular stenosis, tetralogy of Fallot (altogether 8 cases). The origin of the fistula was the noncoronary, right coronary or left coronary sinus (5, 4 and 3 cases) or was not identifiable (1 case). Rupture occurred into the right atrium (6 cases), right ventricle (6) or pulmonary artery (1 case). Repair was undertaken through aortotomy (6 cases), right ventriculotomy (2) or right atriotomy (1) or through aortotomy + right ventriculotomy or atriotomy (4). In one case aortic valve replacement was performed. All survived the operation. Follow-up averaged 9.6 years. Recurrent fistulation, though with small shunt, was found in two cases. Combined two-dimensional and Doppler echocardiography revealed minor cardiac abnormalities in most patients, particularly aortic regurgitation. All the patients were in NYHA function class I or II.
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PMID:Ruptured aneurysm of sinus of Valsalva. Long-term postoperative follow-up. 343 19

Rest and exercise right and left ventricular function were compared using equilibrium gated radionuclide angiography in 19 normal sedentary control subjects (mean age 28 years, range 22 to 34) and 34 patients with hemodynamically documented congenital ventricular septal defect (VSD) (mean age 27 years, range 20 to 40). The 34 patients with VSD were divided into 3 groups: those in Group 1 (17 patients) had pulmonary to systemic blood flow ratios of less than 2 to 1; those in Group 2 (12 patients) had prior surgical closure of VSD (mean interval from surgery 17 years, range 9 to 22), and those in Group 3 (5 patients) had Eisenmenger's complex. Gated radionuclide angiography was performed at rest and during each level of graded supine bicycle exercise to fatigue. Heart rate, blood pressure, maximal work load achieved, and right and left ventricular ejection fractions were assessed. The control subjects demonstrated an increase in both the left and right ventricular ejection fractions with exercise (0.70 +/- 0.07 to 0.79 +/- 0.05 and 0.46 +/- 0.06 to 0.57 +/- 0.04; p less than 0.001 for left and right ventricles, respectively). All study groups failed to demonstrate an increase in ejection fraction in either ventricle with exercise. Furthermore, resting left ventricular ejection fraction in Groups 2 and 3 was lower than that in the control subjects (0.59 +/- 0.09 and 0.54 +/- 0.06 versus 0.70 +/- 0.07; p less than 0.001) and resting right ventricular ejection fraction was lower in Group 3 versus control subjects (0.30 +/- 0.07 versus 0.46 +/- 0.06; p less than 0.001). Thus (1) left and right ventricular function on exercise were abnormal in patients with residual VSD as compared with control subjects; (2) rest and exercise left ventricular ejection fractions remained abnormal despite surgical closure of VSD in the remote past; (3) resting left and right ventricular function was abnormal in patients with Eisenmenger's complex; (4) lifelong volume overload may be detrimental to myocardial function.
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PMID:Rest and exercise ventricular function in adults with congenital ventricular septal defects. 682 41

We describe an unusual case of pulmonary stenosis caused by calcific constrictive pericarditis associated with a congenital ventricular septal defect in a 16-year-old boy who had a 2-week history of progressive dyspnea, cyanosis, fatigue, and bilateral leg edema. Echocardiographic findings led to an initial diagnosis of tetralogy of Fallot; however, findings on chest radiography and CT were suggestive of calcific constrictive pericarditis with pulmonary stenosis, which was then confirmed on cardiac catheterization. Total pericardiectomy and repair of the ventricular septal defect resulted in a satisfactory outcome. Follow-up examinations at 6 and 20 months showed that the patient was asymptomatic and considered to have class I New York Heart Association functional status. To our knowledge, this is the first reported case of calcific constrictive pericarditis with pulmonary stenosis associated with a ventricular septal defect.
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PMID:Idiopathic calcific constrictive pericarditis causing pulmonary stenosis associated with a ventricular septal defect mimicking tetralogy of Fallot. 1269 33

A 26-year-old man had been diagnosed with a cardiac murmur from birth. In 1998, he was admitted to hospital because of slight fatigue. A grade 5/6 continuous murmur was audible near the right sternal border at the second intercostal space. Doppler echocardiography detected an abnormal flow that suggested that an aneurysm of the right coronary sinus of Valsalva had ruptured into the right ventricular inflow tract. Blood tests showed a 19% step-up in oxygen saturation value between the right atrium and right ventricle, indicating a ventricular septal defect with left to right shunt. Coronary angiography revealed a single coronary artery. Surgical repair was carried out and the patient made an uneventful recovery. This rare combination of a ruptured aneurysm of the sinus of Valsalva coexisting with a ventricular septal defect and a single coronary artery has not been reported previously.
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PMID:Ruptured aneurysm of the sinus of valsalva coexisting with a ventricular septal defect and single coronary artery. 1273 91

The authors present a case report of a 12-year-old boy with diagnosis of transposition of the great arteries and ventricular septal defect, who underwent an arterial switch operation plus closure of the septal defect. On follow-up, left ventricular outflow tract obstruction was detected, initially mild. Last year, he started complaining of fatigue on exercise. Severe subaortic stenosis was diagnosed and surgical repair was performed with good results. Subaortic stenosis is a rare complication on follow-up of patients who have undergone an arterial switch operation, particularly those with transposition of the great arteries and ventricular septal defect.
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PMID:Subaortic stenosis following anatomic correction for transposition of the great arteries. 1537 35

We report a case of midterm restenosis of freestyle valved conduit implanted between the right ventricle and the pulmonary artery. A 69-year-old woman visited our hospital with dyspnea and general fatigue and was diagnosed as tetralogy of Fallot. Total corrective surgery including patch closure of ventricular septal defect was performed using the freestyle valve with the Hemashield prothesis to reconstruct the right ventricle to the pulmonary artery. The catheterization revealed almost normal right ventricular pressure on 40th postoperative day, but the opening of the valve was limited. Twenty-two months later she visited our hospital with severe right heart failure. The pressure gradient over the valve was 122 mmHg and the leaflet of the freestyle valve was markedly thickened causing severe stenosis. Use of the freestyle valve for the right ventricular out flow tract reconstruction has to be further studied.
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PMID:[Midterm restenosis of freestyle valved conduit in an adult tetralogy of Fallot]. 1572 71

Late onset of complete heart block is a potentially dangerous complication after open heart surgery for congenital heart disease. The characteristics of patients with late-onset heart block have not been well described. A retrospective review of a pacemaker database was done to identify patients who presented with new onset heart block between 1988 and 2006, after they had been discharged from the hospital after open heart surgery with normal AV conduction. Fifteen patients were identified. The age at the time of the last surgery before the onset of heart block was 2.0 +/- 3.2 years (range: 3 days to 10 years). Nine had a ventricular septal defect repair, four had an atrioventricular canal, and two other patients had other types of heart defect. The last EKG available for analysis before the onset of heart block had been obtained 5.1 +/- 6.5 years (range: 7 days to 16 years) after surgery. The symptoms at the time of presentation were variable. Four patients presented with fatigue or exercise intolerance, two with syncope, two with congestive heart failure, and one with irritability, and the remaining six patients were diagnosed during routine follow-up. The time between open heart surgery and placement of a permanent pacemaker was 6.8 +/- 7.3 years (range: 2 months to 19 years). There were seven patients in whom the onset of heart block was more than 6 years after surgery. Late onset of complete heart block after open heart surgery could be dangerous when presenting without warning. These data would support the notion that patients should be followed for life after repair of congenital heart defects, with special attention to the conduction system, particularly after repair of septal defects.
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PMID:Late onset of heart block after open heart surgery for congenital heart disease. 1776 49

We report a case of multiple signal loss lesions detected by T2* weighted image associated with bacterial endocarditis. A 23 year-old woman, who had undergone surgical closure of ventricular septal defect at age 2, suffered headache and general fatigue after dental treatment. She was admitted to another institution, and examinations revealed bacterial endcarditis. She was transferred to our hospital, and underwent aortic valve replacement. After the valve replacement, CT revealed parenchymal hematoma in the left frontal lobe. CT angiography (CTA) and cerebral angiography (CAG) demonstrated an aneurysm at the distal branch of the left middle cerebral artery. T2* weighted image demonstrated multiple signal loss lesions, which were not revealed by CAG and CTA. We speculated that these signal loss lesions were thrombosed mycotic intracranial aneurysms. She underwent left frontotemporal craniotomy, and the aneurysm was resected. Mycotic aneurysms are rare neurovascular lesions but their mortality and morbidity are high, so careful follow-up examinations are required. T2* weighted image was useful to detect mycotic aneurysms and plan the surgical strategy.
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PMID:[Case of specific MRI T2* weighted image associated with bacterial endocarditis]. 1880 Jun 33


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