Luciano Pasquini

Minimally invasive or interventional repair of atrial septal defects in children : Experience in 171 cases and comparison with conventional strategies

OBJECTIVES The goal of this study was to evaluate percutaneous interventional and minimally invasive surgical closure of secundum atrial septal defect (ASD) in children. BACKGROUND Concern has surrounded abandoning conventional midline sternotomy in favor of the less invasive approaches pursuing a better cosmetic result and a more rational resource utilization. METHODS A retrospective analysis was performed on the patients treated from June 1996 to December 1998. RESULTS One hundred seventy-one children (median age 5.8 years, median weight 22.1 kg) underwent 52 device implants, 72 minimally invasive surgical operations and 50 conventional sternotomy operations. There were no deaths and no residual left to right shunt in any of the groups. The overall complication rate causing delayed discharge was 12.6% for minimally invasive surgery, 12.0% for midline sternotomy and 3.8% for transcatheter device closure (p < 0.01). The mean hospital stay was 2.8 +/- 1.0 days, 6.5 +/- 2.1 days and 2.1 +/- 0.5 days (p < 0.01); the skin-to-skin time was 196 +/- 43 min, 163 +/- 46 min and 118 +/- 58 min, respectively (p < 0.001). Extracorporeal circulation time was 49.9 +/- 10.1 min in the minithoracotomy group versus 37.2 +/- 13.8 min in the sternotomy group (p < 0.01) but without differences in aortic cross-clamping time. Sternotomy was the most expensive procedure (15,000 EUR +/- 1,050 EUR vs. 12,250 EUR +/- 472 EUR for minithoracotomy and 13,000 EUR +/- 300 EUR for percutaneous devices). CONCLUSIONS While equally effective compared with sternotomy, the cosmetic and financial appeal of the percutaneous and minimally invasive approaches must be weighed against their greater exposure to technical pitfalls. Adequate training is needed if a strategy of surgical or percutaneous minimally invasive closure of ASD in children is planned in place of conventional surgery.

Ultrasound tissue characterization detectspreclinical myocardial structural changes inchildren affected by Duchenne muscular dystrophy

Our goal was to identify early changes in myocardial physical properties in children with Duchenne muscular dystrophy (DMDch). Duchenne muscular dystrophy (DMD) is caused by the absence of dystrophin, which triggers complex molecular and biological events in skeletal and cardiac muscle tissues. Although about 30% of patients display overt signs of cardiomyopathy in the late stage of the disease, it is unknown whether changes in myocardial physical properties can be detected in the early (preclinical) stages of the disease. We performed an ultrasonic tissue characterization (UTC) analysis of myocardium in DMDch with normal systolic myocardial function and no signs of cardiomyopathy. Both the cyclic variation of integrated backscatter (cvIBS) and the calibrated integrated backscatter (cIBS) were assessed in 8 myocardial regions of 20 DMDch, age 7 +/- 2 years (range 4 to 10 years), and in 20 age-matched healthy controls. We found large differences in the UTC data between DMDch and controls; the mean value of cvIBS was 4.4 +/- 1.5 dB versus 8.8 +/- 0.8 dB, whereas the mean value of cIBS was 36.4 +/- 7.1 dB versus 26.9 +/- 2.0 dB (p < 10(-6) for both). In DMDch, all eight sampled segments showed cIBS mean values to be significantly higher and cvIBS mean values to be significantly lower than those in the controls. Finally, interindividual differences were greater in DMDch than in controls for both parameters.The myocardium in DMDch displays UTC features different from those in healthy controls. These results show that lack of dystrophin is commonly associated with changes in myocardial features well before the onset of changes of systolic function and overt cardiomyopathy.

Total anomalous pulmonary venous connection: long-term appraisal with evolving technical solutions

OBJECTIVES To evaluate late outcome of non-isomeric total anomalous pulmonary venous connection (TAPVC) repair, controlling for anatomic subtypes and surgical technique. METHODS Between 1983 and 2001, 89 patients (median age 54 days) underwent repair for supracardiac (38), cardiac (26), infracardiac (16) or mixed (nine) TAPVC. Ten patients (11.2%) presented associated anomalies other than PDA. Twenty-eight patients (31.5%) were emergencies, due to obstructed drainage. Supracardiac and infracardiac TAPVC repair included the double-patch technique with left atrial enlargement in 29 patients and side-to-side anastomosis between the pulmonary venous (PV) confluence and the left atrium in 29 patients. Coronary sinus unroofing was preferred for cardiac TAPVC repair. Total follow-up was 727.16 patient-years (mean 8.55 years, 98.8% complete). RESULTS Early mortality was 7.86% (7/89). Ten patients (11.2%) underwent reintervention, including reoperation (eight), balloon dilation (one) and intraoperative stents placement (one), for anastomotic (four) or diffuse PV stenosis (six), with four late deaths. Kaplan-Meier survival is 87.3+/-0.036 SE% at 18.07 years with no difference according to anatomic type or surgical technique. Freedom from PV reintervention for operative survivors is 86.7+/-0.052 SE% at 18.07 years. Cox proportional hazard indicates associated anomalies (P=0.008) and reoperation for intrinsic PV stenosis (P=0.034) as independent predictors of mortality. According to logistic analysis, preoperative obstruction predicts higher risk of reintervention for intrinsic PV stenosis (P=0.022), while the double-patch technique increased the risk of late arrhythmias (P=0.005). CONCLUSIONS Side-to-side anastomosis provides excellent results for TAPVC repair while left atrial enlargement procedures appear to be associated with higher risk of late arrhythmias. Although early and aggressive reintervention for recurrent PV obstruction is mandatory, intrinsic PV stenosis remains a predictor of adverse outcome.

Diagnosis of coronary artery anatomy by two-dimensional echocardiography in patients with transposition of the great arteries.

With the increasing popularity of the Jatene procedure for the treatment of common or D-transposition of the great arteries (D-TGA), the preoperative definition of coronary artery anatomy in D-TGA has assumed great importance. Consequently, the reliability of two-dimensional echocardiography for determining the coronary artery anatomy was studied in 32 infants with D-TGA. Surgical observation of the coronary anatomy was used to assess the accuracy of the echocardiographic diagnosis. The coronary arteries were visualized in 29 of 32 patients (90%), predominantly with the use of parasternal and apical views. In the three remaining patients visualization of the coronary arteries was inadequate to allow determination of their anatomy. The coronary artery anatomy was correctly predicted in 25 of the 29 patients in whom the coronary arteries were visualized. The anatomic patterns included usual coronary anatomy for D-TGA (n = 16), left circumflex coronary from the right coronary artery (n = 6), single right coronary artery (n = 1), single left coronary artery (n = 1), and inverted origin of the coronary arteries (n = 1). The errors in the remaining four patients were (1) false-negative diagnosis of origin of the left circumflex coronary from the right coronary artery (n = 1); (2) false-positive diagnosis of origin of the left circumflex coronary from the right coronary artery (n = 1), and (3) diagnosis of origin of the left circumflex coronary from the right coronary artery when the correct diagnosis was single right coronary artery (n = 2).(ABSTRACT TRUNCATED AT 250 WORDS)

Coronary echocardiography in 406 patients with d-loop transposition of the great arteries

OBJECTIVES The reliability of two-dimensional echocardiography for determining the proximal coronary artery anatomy in d-loop transposition of the great arteries was investigated in 406 infants who underwent surgical repair at one institution. BACKGROUND The origin and proximal course of the main coronary arteries can affect the surgical results of the arterial switch operation. Preoperative determination of the coronary artery anatomy appears to be advantageous for the surgeon. METHODS All infants with d-loop transposition who underwent a two-dimensional echocardiogram and primary surgical repair at our institution between 1987 and 1992 were identified, and the echocardiographic, operative and, when available, autopsy reports were reviewed for coronary artery anatomy, presence of a ventricular septal defect and the spatial relation between the arterial roots. The two-dimensional echocardiographic findings were compared with surgical or autopsy findings. The relation between proximal coronary artery anatomy and 1) a ventricular septal defect, and 2) the spatial orientation of the arterial roots was investigated. Twenty-seven infants diagnosed with an intramural coronary artery were not included because they are the subjects of another report. RESULTS Excluding intramural coronary artery patterns, 10 different types of coronary artery anatomy were seen in these 406 patients. The coronary arteries were imaged adequately in 387 (95%) of the 406 patients. The coronary artery anatomy was determined correctly by two-dimensional echocardiography in 369 (95.4%) of the 387 patients, with 18 errors in diagnosis. During the most recent 2.5 years, 193 (98.5%) of 196 patients were diagnosed correctly, with three diagnostic errors. Patients with a ventricular septal defect or side-by-side great arteries are more likely to have an unusual coronary pattern. CONCLUSIONS Echocardiography appears to be highly reliable for determining proximal coronary artery anatomy in d-loop transposition of the great arteries. An unusual coronary artery pattern is more likely in patients with side-by-side great arteries or posterior aorta or a ventricular septal defect, or both.

Orthotopic Heart Transplantation for Congenital Heart Disease: An Alternative for High-Risk Fontan Candidates?

Objective—Evaluation of incremental risk factors for early mortality in children undergoing orthotopic heart transplantation (OHT) for congenital heart disease. Methods—Between 1988 and 2002, 43 patients (mean age 9.1±7.2 years) underwent 44 OHT for complex TGA (6), DORV (4), single ventricle (21), and other end-stage structural heart disease (11). Two discernible ventricular chambers were present in 18 pts (41.8%). Previous reconstructive or palliative procedures had been previously accomplished in 35 pts (83.3%), including atrial switch (5), systemic-to-pulmonary shunts (10), cavopulmonary anastomosis (9), Fontan completion (6), and others (5). Results—30-day survival for the 2-ventricle subgroup was 94.4±5.4% compared with 67.2±9.5% for the single ventricle subgroup (P =0.04) (overall 78.6%±3.3%). OHT following single ventricle staging to bi-directional cavopulmonary anastomosis exhibited 100% early survival, as opposed to 62.5±17.1% for OHT after systemic-to-pulmonary shunts, and 33.3±19.2% for OHT following failing Fontan (P =0.010). HLHS diagnosis (0.0085) and failing Fontan (P =0.003) were identified as independent predictors of early mortality by regression logistic modeling, while Fontan stage represented the only predictor of overall mortality by Cox proportional hazard. Overall 10-year survival was 54.3±11%. Conclusions—OHT for structural congenital heart disease with single ventricle physiology entails substantial early mortality and bi-directional cavopulmonary anastomosis enables the best transition to heart transplant. OHT should be considered in the decision making process as an alternative to Fontan completion in high-risk candidates, since rescue-OHT after failing Fontan seems unwarranted.

Right oblique subxiphoid view for two-dimensional echocardiographic visualization of the right ventricle in congenital heart disease.

Two-dimensional echocardiography in the right oblique subxiphoid view (ROSV) was used to visualize all right ventricular components in 351 patients younger than 2 years of age (100 normal and 251 with various types of congenital heart disease). The ROSV, which is anterior and parallel to the ventricular septum, displays all right-sided cardiac components, the left atrium and the aorta. This view provided the correct diagnosis in patients with pulmonary stenosis or atresia with intact ventricular septum, Ebsteins anomaly, tricuspid atresia, infundibular ventricular septal defect, tetralogy of Fallot and truncus arteriosus. In all patients studied with the ROSV, an excellent definition of morphologic characteristics, equivalent to that in the right oblique axial angiocardiographic view, was achieved. The ROSV should be used routinely to delineate the right ventricular anatomy in all infants with congenital heart disease.

Two-dimensional echocardiographic anatomy in crisscross heart

The anatomy of crisscross heart was studied in 14 patients, aged 2 days to 26 years, using 2-dimensional (2-D) echocardiography. The visceroatrial situs was solitus in all 14 patients. Crossing of the atrioventricular (AV) valves could be seen in each case by scanning in a subxiphoid or apical 4-chamber view. The subpulmonary infundibulum was deficient in 13 of the 14 patients, resulting in approximation of the pulmonary and tricuspid valves. Subpulmonary stenosis, seen in 11 patients, resulted from proximity of the infundibular septum, the tricuspid valve and the subpulmonary infundibular free wall. A subaortic infundibulum, present in all cases, was well developed in 13 patients, who had malposition of the great arteries, and was short in 1 patient, with nearly normally related great arteries. In cases that conformed to the inverse loop rule (segmental combination (S,D,L) or (S,L,D)), the pulmonary valve was posterior to the tricuspid valve and wedged between the AV valves. In 3 patients (double outlet right ventricle (S,D,L)) the mitral valve straddled into a large, left-sided subaortic infundibulum. In both patients with arterial malposition who did not follow the inverse loop rule, the pulmonary trunk was anterior to the tricuspid valve and well separated from the mitral valve. In patients with crisscross heart the ventricles appeared to have been rotated about their long axes without concomitant motion of the AV valve anuli, producing actual crossing of the ventricular inflow tracts. The right ventricular sinus was significantly smaller and the infundibulum significantly larger in the patients with crisscross heart than in age-matched control subjects.(ABSTRACT TRUNCATED AT 250 WORDS)

New findings concerning cardiovascular manifestations emerging from long-term follow-up of 150 patients with the Williams-Beuren-Beuren syndrome.

AIMS We investigated the prevalence, type, and course of congenital cardiac defects and systemic hypertension in our patients with Williams-Beuren-Beuren syndrome. METHODS AND RESULTS We reviewed the clinical records of all patients with Williams-Beuren syndrome examined between 1981 and 2006. We identified 150 patients, aged from 7 months to 45 years, with a follow-up from 6 months to 25 years, the mean being 6.4 years. A cardiac anomaly was present in 113 of the 150 patients (75%). Defects were typical in over four-fifths of the group. We found supravalvar aortic stenosis in 73 of 113 patients (64.6%), isolated in 43. Pulmonary stenosis, isolated in 18 cases, was found in 51 of 113 (45.1%), while aortic coarctation and mitral valvar prolapse were each found in 7 (6.2%), 3 of the lesions is isolation. Atypical defects were found in 19 patients, tetralogy of Fallot in 2, atrial septal defects in 4, aortic and mitral valvar insufficiencies in 1 each, bicuspid aortic valves in 2, and ventricular septal defects in 9, 4 of the last being isolated. Systemic hypertension, observed in 33 patients (22%), was poorly controlled in 10. Diagnostic and/or interventional cardiac catheterization was undertaken in 24 patients, with 30 surgical procedures performed in 26 patients. Of the group, 3 patients died. CONCLUSION Cardiac defects were present in three-quarters of our patients. Pulmonary arterial lesions generally improved, while supravalvar aortic stenosis often progressed. Atypical cardiac malformations, particularly ventricular septal defects, occurred frequently. Systemic hypertension was found in one-fifth, even in the absence of structural cardiac defects. The short-term mortality was low.

Conal anatomy in 119 patients with d-loop transposition of the great arteries and ventricular septal defect: An echocardiographic and pathologic study

OBJECTIVES We sought to study the range of conal morphology in transposition of the great arteries with ventricular septal defect and their embryologic and surgical implications. BACKGROUND Conal anatomy in transposition of the great arteries and ventricular septal defect is variable and might affect surgical repair. METHODS Conal anatomy was explored using two-dimensional echocardiography in 119 patients with transposition of the great arteries and a large ventricular septal defect who presented between 1984 and 1991. The influence of conal anatomy on surgical technique was determined by review of the operative reports. Specimens of transposition of the great arteries with unusual conal anatomy were selected from the Cardiac Registry for comparison with the echocardiograms. RESULTS One hundred five patients (88.2%) had subaortic conus only with no subpulmonary conus (Group 1). Subarterial conus was present bilaterally in eight patients (6.7%) (Group 2). Four patients (3.4%) had only subpulmonary conus with no (or minimal) subaortic conus (Group 3). Among these four patients, the aorta was posterior to the pulmonary artery in one patient, side by side relative to the pulmonary artery in two patients and slightly anterior in the fourth patient. Subarterial conus was absent bilaterally in two patients (1.7%) (Group 4); the aorta was slightly posterior in one and side by side with the pulmonary artery in the other. CONCLUSIONS This variability of conal anatomy in transposition of the great arteries with ventricular septal defect implies four mechanisms by which transposition can occur. The conal anatomy appeared to affect surgical repair in Groups 1 and 2 insofar as it influenced ventricular outflow tract obstruction. In Groups 3 and 4, an arterial switch operation was performed in four of the six patients. The posterior location of the aorta obviated the need for the Lecompte maneuver in two of these four patients. In the remaining two cases in Groups 3 and 4, the condition was repaired by directing the left ventricular outflow across the ventricular septal defect to the aorta using a patch, with or without placement of a conduit from the right ventricle to the pulmonary artery.