Resetting of the cardiopulmonary baroreflex 10 years after surgical repair of coarctation of the aorta.

OBJECTIVE: To characterise cardiopulmonary baroreflex responses and examine the effects of a 45 minute cycling bout late after successful repair of coarctation of the aorta. SUBJECTS: 10 young adults (mean (SEM) age 18.1 (2.6 years)) operated on for coarctation of the aorta 12.7 (3.5) years earlier, and 10 healthy controls. DESIGN: Forearm blood flow (venous occlusion plethysmography) and vascular resistance, left ventricular internal diastolic diameter, and central venous pressure estimated from an antecubital vein were measured in the supine position at baseline and during five minute applications of lower body negative pressure (LBNP) at -15 mm Hg (LBNP(-15)) and -40 mm Hg (LBNP(-40)). Venous samples were obtained at baseline and during LBNP(-40) for noradrenaline (norepinephrine), adrenaline (epinephrine), renin activity, and aldosterone. The tests were repeated after 45 minutes of moderate exercise. RESULTS: Baseline heart rate (78 (9) v 64 (6) beats/min), echocardiographic cardiac output (6.9 (1.1) v 5.0 (0.2) l/min), shortening fraction (41.7 (1.8)% v 33.3 (1.3)%), and forearm blood flow (3.4 (0.4) v 2.3 (0.3) ml/100 g/min) were higher in the coarctation group than in the controls (p < 0.05). Changes in forearm blood flow and forearm vascular resistance from baseline to LBNP(-40) were similar in both groups, but the relation between forearm vascular resistance and estimated central venous pressure or left ventricular internal diastolic diameter was shifted downward in the coarctation group. Plasma adrenaline was increased in the coarctation group (baseline: 3.2 (0.6) v 2.4 (0.3) pmol/l in controls; LBNP(-40): 687 (151) v 332 (42) pmol/l) (p < 0.05). Both groups showed a similar downward displacement of forearm vascular resistance (p < 0.05) after exercise. CONCLUSIONS: There appears to be resetting of the cardiopulmonary baroreflex to a lower forearm vascular resistance in young adults operated on for coarctation of the aorta, associated with hyperdynamic left ventricular function. Raised circulating adrenaline could contribute to the lower forearm vascular resistance.

Influence of the postoperative period and surgical procedure on ambulatory blood pressure-determination of hypertension load after successful surgical repair of coarctation of the aorta.

AIMS: This study quantified hypertension load using 24-h ambulatory blood pressure monitoring after successful repair of coarctation of the aorta less than (1) or more than 10 years previously (2) and examined the influence of the surgical procedure (anastomosis or subclavian flap). METHODS AND RESULTS: Ambulatory blood pressure recordings were obtained using an Accutracker II monitor every 30 min during the day and hourly, at night. Day and night systolic and diastolic values were higher in coarctation of the aorta than in controls: (day: systolic blood pressure/diastolic blood pressure: 133/71 +/- 6/4 vs 115/66 +/- 3/2 night: systolic blood pressure/diastolic blood pressure: 117/61 +/- 4/4 vs 107/57 +/- 3/2 mmHg, P < 0.01) and at all times, were higher in coarctation of the aorta (2) than in coarctation of the aorta (1). Clinical daytime systolic hypertension was observed in 20% of recordings from coarctation of the aorta (1) and 49% from coarctation of the aorta (2) while diastolic hypertension was not observed. However, systolic blood pressure and diastolic blood pressure responses to daily activities were significantly higher in coarctation of the aorta than in controls and this was more marked in coarctation of the aorta (2) than in coarctation of the aorta (1). Type of surgery did not affect either hypertension prevalence or blood pressure reactivity. CONCLUSIONS: These observations indicate exaggerated systolic blood pressure and diastolic blood pressure reactivity after repair of coarctation of the aorta, the prevalence of systolic hypertension doubling 10 years after surgery.

Peripheral blood flow responses to exercise after successful correction of coarctation of the aorta.

OBJECTIVES: The purpose of this study was to characterize peripheral flow kinetics in response to progressive discontinuous maximal exercise in 10 patients who underwent repair of coarctation of the aorta and 11 age-matched healthy adolescents. BACKGROUND: An impairment of leg blood flow has been suggested on the basis of exaggerated femoral muscle lactate accumulation in patients with successful repair of coarctation. Few data are available describing blood flow kinetics of the exercising leg in such patients. METHODS: Duplex ultrasound provided transcutaneous measurements of peak systolic and end-diastolic flow velocities of the femoral, humeral and renal arteries at rest and immediately after mild, moderate and maximal exercise intensities for computation of mean velocity, resistance index and femoral blood flow. RESULTS: Femoral mean velocity and femoral blood flow increased linearly with exercise intensity in both groups, but the slope of this increase was significantly lower in patients. Similarly, humeral mean velocity increased significantly less in patients than in control subjects. Femoral resistance index sharply decreased from that at rest (patients [mean +/- SE] 1.4 +/- 0.04; control subjects 1.4 +/- 0.03) to mild exercise intensity in both groups (patients 0.69 +/- 0.03; control subjects 0.72 +/- 0.03). A further decrease was observed at maximal exercise in patients (0.60 +/- 0.04, p = 0.08) but not in control subjects (0.69 +/- 0.02). CONCLUSIONS: These observations suggest that despite a greater exercise-induced femoral vasodilation, patients with successful correction of coarctation of the aorta demonstrate an impaired lower limb blood flow in response to strenuous dynamic exercise. In the absence of stenosis at rest, this alteration could result from exaggerated flow turbulence in the descending aorta distal to the site of correction because of loss of elasticity at the site of the resection of the coarcted segment.

Total anomalous pulmonary venous return complicating portoenterostomy for biliary atresia.

Cardiovascular anomalies such as absent inferior vena cava and preduodenal portal vein are reported in cases of biliary atresia and make hepatic portoenterostomy a technical challenge. The authors present the case of a severe cardiac anomaly that significantly altered the functional outcome of a Kasai procedure. Baby M., an 8-week-old boy born with total anomalous pulmonary venous return (TAPVR), underwent hepatic portoenterostomy for biliary atresia. Over the next 3 months he remained icteric and febrile, and failed to gain weight. After multiple antibiotic treatments for suspected cholangitis, he underwent reexploration of the portoenterostomy, with no improvement in his overall condition. His prognosis was considered dismal because correction of the cardiac anomaly is associated with a high mortality rate (> 90%). The cardiac surgeon agreed to attempt a cure of the TAPVR, provided liver transplantation is contemplated if the patient survived. Within 48 hours postoperatively, his hepatic function had improved drastically. He became afebrile, had an improved appetite and weight gain, and was finally discharged 203 days after admission. One year later, he is thriving and remains anicteric. The exact reason for this drastic improvement is not well understood, but the right-sided cardiac failure caused by the TAPVR had a significant effect on the functional outcome of the portoenterostomy.

Survival analysis of infants under age 18 months presenting with tetralogy of Fallot.

The trend to perform early primary repair of tetralogy of Fallot prompted us to review our experience in the current era with the traditional protocol consisting of palliation during infancy, if required, and repair after infancy. During a 10-year period, 270 infants with tetralogy of Fallot presented aged less than 18 months. Thirteen infants (4.8%) had major noncardiac lesions that precluded definitive care for their congenital heart disease. Twenty infants (7.4%) had major associated cardiac lesions (atrioventricular septal defect or absent pulmonary valve syndrome, or both). Survival in this group was poor, with only 58% +/- 12% reaching the age of 10 years. Four of the seven deaths occurred before intracardiac repair was performed. The remaining 237 infants presented with isolated tetralogy of Fallot. Eight-nine percent +/- 2.3% survived to age 10 years. Sixty percent of these infants required palliation, and survival in these infants did not differ from that in those who never required palliation. However, 19 infants (8%) required palliation in the first month of life. In these children, survival to age 10 years was significantly lower (77%), secondary palliation was frequently required (n = 11), and a transannular patch or conduit at the time of repair (10 of 14 patients) was more likely needed than it was in children who had not undergone a palliative procedure during the neonatal period. The survival in infants with tetralogy of Fallot is unlikely to be different, regardless of whether primary repair or a staged repair is carried out. The quality of survival, including the exercise capability and absence of arrhythmias, must be assessed to determine which protocol is superior.

[Critical aortic stenosis in newborn infants. Twenty-five years' experience]. / Sténose aortique critique du nouveau-né. Expérience de vingt-cinq ans.

Operative mortality for critical aortic stenosis in newborn having been reported as high as 20-80%, the percutaneous aortic valvuloplasty is looked upon as an alternative therapy. We elected to review our surgical experience in the last 25 years before changing our therapeutic approach. Among 37 infants included in the study, 28 were boys and 9 girls. Mean age at diagnosis was 14.5 days (1 to 113 days) and decreased to 4.1 days in the last 5 years. Surgery was done at a mean of 37.2 days; 22 infants were operated on before one month of age and 9 in their first week of life. Five died from ventricular fibrillation at incision. Trans-ventricular valvulotomy was attempted in 4 and remaining 28 had trans aortic valvuloplasty. The overall survival of 40% had improved in last five years to 75%. All patients with trans-ventricular approach died and also did the only child with percutaneous angioplasty. Only 13% patients weighing less than 3 kg survived the operation. Follow-up was of 93.3% with a mean time of 66 months. All but one are in NYHA class 1 or 2. Our study identified the following risk factors: preoperative hemodynamic state, surgical weight, associated anomalies of the left ventricle, transventricular valvulotomy and the year of surgery. In view of improved survival, surgery remains a good therapeutic choice.

Acute necrosis of the intestinal mucosa with high serum levels of diamine oxidase.

A patient with acute necrosis of the intestinal mucosa and high serum diamine oxidase activity is described. The 71-year-old woman, with a history of hypertension and cardiovascular and peripheral arteriosclerotic disease, presented with acute epigastric pain, vomiting, and a deteriorating hemodynamic condition. Serum level of the intestinal enzyme diamine oxidase (DAO) obtained on admission, approximately 24 hr after the onset of symptoms, was 7.4 times above the normal value. An exploratory laparotomy performed 6 hr later revealed cyanosis and areas of transmural necrosis involving the entire small bowel. The bowel was not resected because of the extent of lesion. Thirty hours after the first sample was taken and 2 hr before death, the serum DAO level was only slightly above normal. It is suggested that this biochemical marker could provide a valuable tool for the early diagnosis of intestinal ischemia.