Claude Dupuis

The “adult” form of the scimitar syndrome

One hundred twenty-two cases of the adult form of the scimitar syndrome were collected from different cardiologic centers. The clinical, radiographic and hemodynamic findings are described. The scimitar syndrome is defined as an anomalous right pulmonary venous drainage, partial or complete, to the inferior vena cava. Additional characteristics of this syndrome such as hypoplasia and abnormalities of the vascular supply to the right lung, dextrocardia and abnormalities of the bronchial segmentation are common; bronchiectases are rare. The left to right shunt was less than 50% in 100 of the 122 patients. The pulmonary arterial pressures were normal in 94 patients and slightly elevated in 28. A follow-up study of these patients showed that, without surgical correction, they lead a normal life. An awareness of this syndrome may avoid unnecessary invasive diagnostic procedures and surgical treatment for most patients.

Infantile form of the scimitar syndrome with pulmonary hypertension

Twenty-five newborns and infants aged < 1 year with the scimitar syndrome and pulmonary hypertension from 12 European pediatric centers were examined. Cardiac failure and severe respiratory insufficiency were always present. In 23 cases, pulmonary hypertension was due to a large shunt between abnormal arteries originating from the abdominal aorta and supplying the lower part of the right lung (vascular sequestration). In the last 2 cases, pulmonary hypertension was secondary to stenosis of the common trunk of the right pulmonary veins. Three of 10 patients who received only medical treatment survived; 2 are doing well, with pulmonary arterial pressures that have returned to normal, and the other had severe residual pulmonary hypertension. Six of 15 patients who underwent surgery survived. There were 5 ligations of a patent ductus arteriosus with 5 deaths, 3 pulmonary resections with 2 deaths, 1 dilation of a tight stenosis of the common trunk of the right pulmonary veins with 1 death, and 6 ligations of the abnormal arterial vessels with 5 surviving patients who are in good condition. Ligation of the abnormal arterial vessels appears to be the best type of treatment.

Percutaneous transluminal balloon valvuloplasty of congenital pulmonary valve stenosis, with a special report on infants and neonates

Fifty-six percutaneous transluminal balloon valvuloplasty procedures were performed in 51 patients suffering from congenital pulmonary valve stenosis. The patients ranged in age from 1 day to 60 years (mean 6.9 years); 21 were infants less than 1 year of age, including 8 neonates. The peak systolic pressure gradient was greater than 50 mm Hg (mean 81.5) in 47 cases, and less than 50 mm Hg (mean 34.6) in 9. Valvuloplasty enabled a reduction in the mean right ventricular peak systolic pressure from 93.1 to 51.6 mm Hg (p less than 0.001), the mean transvalvular gradient from 73.4 to 27.0 mm Hg (p less than 0.001) and the mean right ventricular pressure expressed as a percent of systemic pressure from 99.5 to 52.0% (p less than 0.001). In infants and neonates, the mean right ventricular pressure expressed as a percent of systemic pressure decreased from 117.3 to 64.4% (p less than 0.001). In 23 patients, follow-up cardiac catheterization at 1 to 17 months revealed a significant change in the right ventricular systolic pressure, which decreased from 54.0 to 46.7 mm Hg (p less than 0.05), and in the peak systolic pressure gradient, which decreased from 27.3 to 22.6 mm Hg (p less than 0.05). Valvuloplasty is an effective procedure in relieving pulmonary stenosis in patients of all ages, including neonates.

Bacterial pericarditis in infancy and childhood

The authors report on a cooperative study of 43 cases of bacterial pericarditis observed in children. This disorder was suspected in patients with septicemia who developed symptoms and signs of pericarditis (precordial pain, muffled heart sounds, pericardial friction rub, cardiomegaly). Early diagnosis of this condition is now facilitated by echocardiography. A combination of medical and surgical treatments (appropriate antibiotic therapy after culture and sensitivity tests and early pericardial drainage) led to complete recovery in almost all of the cases (42 of 43). After long-term follow-up, no cases of constrictive pericarditis were observed.

Asymptomatic form of left pulmonary artery sling

Eight patients with left pulmonary artery sling, which were asymptomatic at the time of the last consultation, are described: 2 adults and 1 child with no history of symptoms, 3 children with mild forms of airways obstruction and 2 patients with typical severe symptoms of airways obstruction in infancy. The mean follow-up of these 8 patients was 10 years (range 4 to 23), and in 1986, all were in good health and free of respiratory symptoms. The long-term prognosis is usually good.

Percutaneous balloon angioplasty in an infant with obstructed total anomalous pulmonary vein return

This is a report of successful dilation of stenosis of the common trunk in a case of total anomalous pulmonary vein return into the left superior vena cava in a 3 month old infant. Percutaneous angioplasty was performed with a 6 mm diameter balloon catheter. Right ventricular systolic pressure decreased from 96 to 60 mm Hg, mean pulmonary vein pressure decreased from 26 to 14 mm Hg and left ventricular systolic pressure increased from 70 to 90 mm Hg.

Severe congestive heart failure in newborns due to giant cutaneous hemangiomas

Abstract Cutaneous hemangiomas of the head and the neck are not rare, but they seldom present more than a cosmetic problem. In the newborn, congestive heart failure (CHF) is a well known complication of arteriovenous fistulas located in the brain or the liver but have rarely been observed with such cutaneous hemangiomas. We report 2 new cases of severe CHF and point out the dramatic improvement after embolization undertaken in 1 patient.

Influence of clinical and hemodynamic characteristics on signal-averaged electrocardiogram in postoperative tetralogy of Fallot

This study examines the relation between signal-averaged electrocardiographic measurements and the occurrence of spontaneous ventricular arrhythmias in 86 patients with a postoperative right bundle branch block after repair of tetralogy of Fallot; special attention was given to the influence of age, body surface area and right ventricular systolic pressure on signal-averaged electrocardiograms. Twenty-eight of the 86 patients had significant ventricular arrhythmias on 24-hour ambulatory monitoring. A positive linear correlation was found between filtered QRS duration and age at evaluation or body surface area (r = 0.45, p = 0.00001; r = 0.54, p < 0.00001, respectively) and between amplitude of the last 40 ms of the filtered QRS and right ventricular systolic pressure (r = 0.48, p < 0.001). A negative linear correlation was found between amplitude of the last 40 ms of the filtered QRS and age at evaluation or body surface area (r = -0.27, p = 0.01; r = -0.34, p = 0.002, respectively). When the age of the patients or the body surface area was considered with an analysis of covariance, the presence of ventricular arrhythmias was associated with a higher amplitude of the last 40 ms of the filtered QRS. In addition, an amplitude of the last 40 ms of the filtered QRS > 170 microV had an excellent sensitivity (100%) and a good specificity (88%) for identifying patients with both right ventricular systolic hypertension and spontaneous ventricular arrhythmia. Thus, adjustment of signal-averaged parameters for age at evaluation and body size is mandatory when studying postoperative tetralogy of Fallot.

Isolated ventricular discordance and complete atrioventricular canal in situs inversus: Report of successful surgical repair

Isolated ventricular discordance is a very rare malformation, most often lethal in the first months of life. A patient with this lesion survived to age 9 years in spite of the coexistence of a complete form of atrioventricular (A-V) canal. Surgical repair with a Mustard procedure was associated with correction of the endocardial cushion defect. Situs inversus itself caused no problem. Although permanent complete A-V block occurred, the result has been satisfactory so far. This case is thought to be the first successful correction of this malformation.