Albert P. Rocchini

Coronary Artery Fistulas in Infants and Children: A Surgical Review and Discussion of Coil Embolization

BACKGROUNDnCoronary artery fistula (CAF) is a rare congenital anomaly that can be complicated by intracardiac shunts, endocarditis, myocardial infarction, or coronary aneurysms. Recent reports have emphasized the efficacy of percutaneous transcatheter techniques. The purpose of this article is to review a 28-year surgical experience with CAF as a standard for comparison and to discuss the emergence and efficacy of transcutaneous catheter coil embolization as an alternative form of therapy.nnnMETHODSnFrom 1968 to 1996, 17 patients (age, 6 weeks to 16.5 years; mean age, 5.5 years) were diagnosed with CAF: 8 of 12 by echocardiography and 17 of 17 by cardiac catheterization. All patients with isolated CAF (n = 13) were asymptomatic despite significant clinical, electrocardiographic, and chest roentgenographic findings in 10. Sixteen had congenital CAF and 1 had acquired CAF after tetralogy of Fallot repair with injury of the anomalous left anterior descending coronary artery. Associated anomalies included tetralogy of Fallot (2), atrial septal defect (1), and patent ductus arteriosus (1). Nine fistulas originated from the right coronary artery and eight from the left. Drainage was to the right ventricle (9), right atrium (4), pulmonary artery (3), and left atrium (1).nnnRESULTSnAll patients had a median sternotomy with epicardial or endocardial ligation. Cardiopulmonary bypass was used in 8; 1 of these (iatrogenic CAF) required distal internal mammary artery bypass graft. There were no operative or late deaths. Follow-up evaluation by physical examination (17), echocardiography (8), and catheterization (2) showed no evidence of recurrent or residual CAF. A retrospective review of the 16 available cine cardioangiograms showed that coil embolization was possible in, at most, 6 patients.nnnCONCLUSIONSnEarly surgical management of CAF is a safe and effective treatment resulting in 100% survival and 100% closure rate. Transcatheter embolization is a reasonable alternative to standard surgical closure in only a very small, select group of patients. These surgical results should be considered the standard against which transcatheter techniques are compared.

Balloon aortic valvuloplasty: Results of the Valvuloplasty and Angioplasty of Congenital Anomalies Registry

Data from 204 children and infants who underwent aortic balloon valvuloplasty between 1982 and 1986, reported to the Valvuloplasty and Angioplasty of Congenital Anomalies Registry, were reviewed. Valvuloplasty was successful in 192 of 204 children, reducing the peak systolic left ventricular ejection gradient from 77 +/- 2 to 30 +/- 1 mm Hg, p less than 0.001. The same degree of aortic stenosis gradients reduction was noted in both the 38 children under 1 year of age and in the 166 children over 1 year of age. Significant complications included death, aortic regurgitation and femoral artery thrombosis or damage. The incidence of these complications correlated with the age of the child, the ratio of valvuloplasty balloon size/anulus size, or both. The data suggest that percutaneous balloon valvuloplasty provides effective acute relief of valvar aortic stenosis in both infants and children. However, long-term follow-up data are necessary before balloon valvuloplasty can be established as a treatment of choice for congenital valvar aortic stenosis.

Relationship between insulin resistance and abnormal lipid profile in obese adolescents

OBJECTIVEnTo determine whether the lipid abnormalities observed in obese adolescents are associated with insulin resistance.nnnMETHODSnWe evaluated the relationship between lipid levels and insulin resistance in 82 obese adolescents. Insulin resistance was assessed by fasting insulin level and sum of the insulin values after an oral glucose tolerance test in all 82, and were compared with data from 40 nonobese adolescents. Whole-body glucose uptake during euglycemic hyperinsulinemia (M value) was performed in 19 of the obese adolescents and compared with that of 24 nonobese young adults.nnnRESULTSnThe obese adolescents had significantly elevated low-density lipoprotein cholesterol (LDL-C) (3.09 +/- 0.73 mmol/L; 119 +/- 28.2 mg/dl) and triglycerides (1.22 +/- 0.62 mmol/L; 108 +/- 54.6 mg/dl) and low high-density lipoprotein cholesterol (HDL-C) levels (0.94 +/- 0.24 mmol/L; 36 +/- 9.1 mg/dl) when compared with values in the nonobese subjects. M values were significantly depressed in the obese compared with the nonobese subjects. Adiposity significantly correlated with low HDL-C and elevated triglyceride values. From the variables representing insulin resistance, the strongest correlation with the abnormal lipid profile was found for the M value. A stepwise multiple regression analysis revealed that the M value was the only step entered into the relationship for triglycerides and LDL-C, and both M value and fasting insulin were entered for HDL-C.nnnCONCLUSIONnIn obese adolescents the degree of insulin resistance explains a significant portion of the variance in the levels of triglycerides, LDL-C, and HDL-C.

Exercise-Induced Hypertension After Surgical Repair of Coarctation of the Aorta

To study exercise-induced hypertension after surgical repair of coarctation of the aorta, 30 patients aged 6 to 30 years (median 14) were exercised to exhaustion on a treadmill using the Bruce protocol. Twenty patients with hemodynamically insignificant heart disease served as controls. Systolic blood pressure was measured simultaneously in the arm and leg before and immediately after exercise. There was no significant difference between the control and coarctectomy groups with regard to age at exercise, duration of exercise, maximal heart rate or serum lactate after exercise. Blood pressure in the arm increased significantly after exercise in both groups (control group: 110 [range 90 to 140] to 135 [range 114 to 164] mm Hg, P P P P P r = 0.91, P This high correlation suggests that the residual obstruction across the site of the coarctectomy causes the observed postexercise systolic hypertension in the arm. Exercise testing with blood pressure measurements should be performed in all children after repair of coarctation of the aorta. In those whose postexercise systolic pressure in the arm exceeds 200 mm Hg, catheterization should be considered to measure central aortic pressure with exercise because reoperation, antihypertensive medication or restriction of severe physical effort may be necessary.

Impact of arrhythmia circuit cryoablation during Fontan conversion for refractory atrial tachycardia

Refractory atrial arrhythmias in late postoperative Fontan patients are usually associated with residual hemodynamic abnormalities and result in significant morbidity and mortality. Surgical revision of the Fontan anastomosis may improve hemodynamics without eliminating tachycardia. This study sought to assess the impact of surgical cryoablation of the arrhythmia circuit at the time of Fontan conversion on the clinical recurrence of tachycardia. Sixteen consecutive atriopulmonary Fontan patients with refractory atrial arrhythmias underwent surgical conversion to lateral tunnel total cavopulmonary anastomosis (15) or Fontan revision (1 patient). The initial 4 patients underwent Fontan conversions alone, without specific arrhythmia surgery. The subsequent 12 patients underwent electrophysiologically guided cryoablation of the tachycardia circuits at the time of surgical conversion. The mean age at Fontan revision was 15.6 +/- 3.8 years. Cryoablation was directed to 3 identified major tachycardia circuits: the inferomedial right atrium, the superior rim of the prior atrial septal defect patch, and along the lateral right atrial wall. Transmural antitachycardia pacemakers were implanted in 11 of the 16 patients. There was no surgical mortality in either group, and all patients improved in functional classification. All patients not undergoing cryoablation experienced recurrent symptomatic tachycardia requiring antiarrhythmic therapy (median follow-up, 54 months) versus 2 of 12 patients receiving cryoablation (median follow-up, 25 months; p <0.02). Thus, surgical cryoablation of the arrhythmia circuit at the time of Fontan conversion is highly effective in the management of refractory atrial arrhythmias, and is superior to Fontan conversion alone.

Pathogenesis of paradoxical hypertension after coarctation resection.

SUMMARY The pathogenesis of paradoxical hypertension after resection of coarctation of the aorta was investigated by comparing the course of seven children undergoing repair of coarctation with five acyanotic children undergoing elective cardiovascular surgery. During the first 24 hours after surgery, all coarctation patients demonstrated a rise in systolic blood pressure (35 ± 15.5 mm Hg; P < 0.001), a significant depression in cold pressor test response, and only a slight elevation in plasma renin activity. In the next 24–72 hours, coarctation patients developed a rise in diastolic blood pres- sure (26.8 ± 10.6 mm Hg; P < 0.001), plasma renin activity (22.9 ± 10.2/ml/hr; P < 0.001) and fluid retention. By contrast, control patients had no significant postoperative changes. Abdominal pain occurred in five coarctation patients during the period of maximal plasma renin activity. The data suggest that the sympathetic nervous system may be responsible for the initial phase of hypertension after coarctation resection and that the renin angiotension system plays a major role in the second phase of hypertension and in the pathogenesis of mesenteric arteritis.

Left ventricular diastolic filling in children with hypertrophic cardiomyopathy: assessment with pulsed Doppler echocardiography.

Altered left ventricular filling patterns in hypertrophic cardiomyopathy have been demonstrated by M-mode echocardiographic and radionuclide techniques. Because pulsed Doppler ultrasound provides the capability to directly measure blood flow velocity across the mitral valve, it was hypothesized that this technique would be useful for demonstrating left ventricular filling abnormalities. Simultaneous Doppler ultrasound examination of the left ventricular inflow, M-mode echocardiograms and phonocardiograms were performed in 17 children and young adults: 10 with hypertrophic cardiomyopathy (aged 6 to 20 years) and 7 with a normal heart (aged 10 to 18 years). From the Doppler studies, measurements of various diastolic time intervals, peak flow velocity during rapid filling (E velocity) and peak flow velocity during atrial contraction (A velocity) were made. Several areas within the Doppler flow envelope were calculated: first 33% of diastole (0.33 area), first 50% of diastole, triangle under the E velocity (E area) and triangle under the A velocity (A area). These were expressed as a percent of area under the total flow envelope. From the M-mode studies, left ventricular endocardial echoes were digitized and peak rates of increase in left ventricular dimension were determined and normalized for end-diastolic dimension. Diastolic time intervals, including isovolumic relaxation time, were calculated using the phonocardiogram to determine end-systole. The E velocity was lower (0.71 +/- 0.23 versus 0.91 +/- 0.11 m/s, p less than 0.05), 0.33 area/total area was less (0.46 +/- 0.11 versus 0.58 +/- 0.08, p less than 0.05) and the isovolumic relaxation time was prolonged (56 +/- 2 versus 31 +/- 1 ms, p less than 0.05) in patients with hypertrophic cardiomyopathy.(ABSTRACT TRUNCATED AT 250 WORDS)

Surgical repair of the congenitally malformed mitral valve in infants and children

BACKGROUNDnMitral valve remodeling techniques were applied to 26 infants and children (mean age, 6.0 years, range, 0.4 to 15.9 years) with various forms of congenital mitral valve disease over a 7-year period. Patients with atrioventricular canal, L-transposition and single ventricle were excluded. Intraoperative transesophageal echocardiography (TEE) was utilized to assess the repair and guide the need for immediate intervention.nnnMETHODSnTwenty-one patients had mitral regurgitation: 10 with cleft anterior mitral leaflet, 7 with annular dilatation, 1 with normal leaflets with an obstructing cord, 2 with prolapsed leaflets and elongated cords, and 1 with restricted leaflet motion, normal papillary muscles, and shortened cords. Of the 5 mitral stenosis patients, 3 had supravalvular mitral ring, 1 had midvalvular mitral ring, and 1 had a parachute valve. Three of the mitral stenosis patients had additional stenotic lesions. Concurrent repair of associated lesions was performed in 21 patients (78%).nnnRESULTSnOperative mortality was 3.8% (n = 1). There were no late deaths. Immediate rerepair in 4 patients resulted in improved function. All mitral stenosis patients improved. A total of 20 mitral regurgitation patients (95%) improved; 1 required mitral valve replacement. Mean follow-up is 31 months (range, 2 to 81 months). All patients are in New York Heart Association functional class I or II.nnnCONCLUSIONSnMitral valve repair can be successfully performed in infants and children with excellent short- and midterm results. Assessment using transesophageal echocardiography can guide the necessity for immediate rerepair to achieve improved function.

Experience with Valved Conduits for Repair of Congenital Cardiac Lesions

Dacron valved conduits or aortic allografts were placed between the right heart and the pulmonary artery for repair of various complex congenital cardiac anomalies in 56 patients (aged 15 days to 33 years; median, 11 years). Forty-four patients had a total of 56 previous palliative procedures, which contributed to postoperative morbidity and mortality. Six patients had a total of seven episodes of early or late sepsis involving the conduit. One patient, treated for early sepsis, again developed infection in the Hancock graft 1 year postoperatively and died. Three other patients, 2 with calcified allografts, developed infections 4 months to 7 years following repair and required graft replacement. Hemodynamic data 1 month to 5 years (mean, 1.6 years) following repair revealed mild to moderate obstruction (less than 45 mm Hg gradient) at the Hancock conduit valve ring in 13 of 19 patients, while 5 had large pressure gradients (greater than 75 mm Hg). All aortic allografts had severe obstruction and calcification necessitating graft replacement. It is anticipated that improved technique and appropriate timing of palliative and corrective operations will substantially reduce or eliminate these problems.

Taussig-Bing anomaly: Arterial switch versus Kawashima intraventricular repair

BACKGROUNDnCurrent corrective surgical approaches for the Taussig-Bing heart include arterial switch with ventricular septal defect (VSD) closure and intraventricular repair as described by Kawashima.nnnMETHODSnBetween 1983 and 1994, 20 children underwent intracardiac repair of Taussig-Bing anomaly. Mean age at operation was 17 months (range, 1 week to 9 years). Prior palliation included pulmonary artery band (15) with coarctation repair (8) and atrial septectomy (1). Arterial switch with VSD closure was performed in 16 patients, 10 with anteroposterior great arteries. Kawashima repair was performed in 4 patients, all with side-by-side great arteries.nnnRESULTSnAfter arterial switch, there was one operative death (6.2%) due to myocardial ischemia and three late deaths (18.7%) due to pulmonary hypertension, gastrointestinal bleeding, and acute lymphocytic leukemia. In the Kawashima repair group there have been no deaths. After arterial switch, 9 patients underwent 11 reoperations for residual coarctation (3), residual pulmonary artery stenosis (2), aortic valve replacement, aortic valvuloplasty, unrecognized VSD, mitral valvuloplasty, mediastinitis, and pacemaker insertion. After Kawashima repair, 1 patient underwent reoperation for baffle stenosis and 1 for an unrecognized VSD.nnnCONCLUSIONSnFor children with Taussig-Bing anomaly, the Kawashima intraventricular repair (for side-by-side great arteries) preserves the native aortic valve and avoids coronary dissection. The arterial switch operation with VSD closure can be applied without ventriculotomy to all great artery relationships and allows neonatal repair with or without concomitant coarctation repair. Both techniques yield excellent early and intermediate-term results despite the high rates of prerepair palliation and postrepair reoperation for both groups.