Complete atrioventricular canal and total anomalous pulmonary venous drainage: a rare association.
Explore the source record for details and available documents.
Biomedical subjects
Publications and source records attributed to C Marcelletti.
Explore the source record for details and available documents.
A 2.5-year-old boy with a diagnosis of situs solitus, tricuspid atresia, anatomically corrected malposition of the great arteries (S,D,L), left juxtaposition of atrial appendages, and pulmonary stenosis underwent successful total cava-to-pulmonary connection by means of a superior vena cava-to-pulmonary artery end-to-side anastomosis associated with an inferior vena cava-to-pulmonary artery direct anastomosis. Anatomic features and surgical technique are described.
Explore the source record for details and available documents.
BACKGROUND: The bidirectional cavo-pulmonary anastomosis is a useful surgical procedure for patients with complex congenital heart disease characterized by univentricular physiology. This operation significantly increases effective pulmonary blood flow and rarely causes distortion of the main pulmonary arterial branches. The aim of this study was to evaluate the clinical and pathophysiologic consequences of bidirectional cavo-pulmonary anastomosis as well as the effectiveness of this surgical approach as a definitive palliation. METHODS: Clinical, hemodynamic and functional data of 12 patients who had undergone a bidirectional cavo-pulmonary anastomosis were reviewed. Each patient underwent clinical examination, chest x-ray, ambulatory EKG, bidimensional color-Doppler echocardiography, spirometric analysis, lung perfusion scintigraphy, cardiac catheterization and angiography, and stress test. Stress test data were compared to those obtained by a normal population of age-matched children. RESULTS: After a mean follow-up interval of 28 +/- 20 months all patients showed a good clinical condition. Improved oxygenation, exercise tolerance and growth velocity were noted in all patients. Only 34% of patients showed abnormalities of chest x-ray, 29% manifested significant arrhythmias during ambulatory EKG and 20% showed abnormal ventricular function echocardiographically. Spirometry was normal in all patients, while lung perfusion scintigraphy was constantly abnormal with right/left and upper/lower perfusion mismatch. At cardiac catheterization a reduction of end diastolic volume (from 106.9 +/- 50.6 to 67.3 +/- 41.6 ml, p < 0.05) and end systolic volume (from 58.0 +/- 27.9 to 32.5 +/- 33.5 ml, p < 0.05) was noted. The functional evaluation of these patients was highly abnormal due to an impaired response to effort as evidenced by work time (p < 0.0001 vs normal) and stress-induced cardiovascular modifications. CONCLUSIONS: On the basis of these findings, we suggest that: 1) bidirectional cavo-pulmonary anastomosis is useful as an intermediate step towards a Fontan procedure, as it improves the hemodynamic performance of the systemic ventricle; 2) conversely, bidirectional cavo-pulmonary anastomosis should not be considered a form of definitive palliation for complex congenital heart disease because in these patients the response to exercise remains unsatisfactory.
Ventricular hypertrophy is a recognized risk factor for the Fontan operation in cases of complex cardiac anomalies with unrestricted pulmonary blood flow and subaortic obstruction. Between 1986 and 1991 we have treated 23 such patients with a new type of palliation combining a main pulmonary artery-ascending aorta anastomosis with a bidirectional cavopulmonary anastomosis. Their ages averaged 57 +/- 36 months (7 to 155 months). Nine patients had (S,L,L) single ventricule with transposition and the other 14 had more complex cardiac anomalies unsuitable for biventricular repair. Subaortic obstruction was defined by a hemodynamic criterion (n = 6) or by a morphologic criterion (n = 17). There were five hospital deaths (21.7%): All three patients older than 7 years of age died (p = not significant). Follow-up averaged 28 +/- 21 months (range 1 to 58 months). Among the 18 hospital survivors, the proportions surviving 1 and 5 years after the operation were 78% and 63%, respectively. Control cardiac catheterization in 11 patients showed no or trivial subaortic gradient, a mean cavopulmonary pressure of 10 +/- 3 mm Hg, and a mean arterial oxygen saturation of 83%. Nine patients underwent secondary Fontan repair in our institution 21 +/- 4 months after palliation, without deaths. Another patient died elsewhere, 3.7 years after palliation, as a result of hemorrhage at sternal reentry during attempted Fontan operation (overall mortality at repair, 10%). The other eight patients are awaiting Fontan operation. This staged approach reduces both pressure and volume ventricular load and provides adequate oxygenation before the Fontan operation. In our experience, it resulted in reduced mortality at definitive repair.
We studied 60 patients who had survived the Mustard procedure for transposition of the great arteries, performed between the ages of 2 days and 24 months (mean 4.51 +/- 3.79). All patients were given a postoperative 24-hour dynamic electrocardiogram at 15 days, 1 year and 3 years and then every 2 years. Those who were found to have sinus node dysfunction during follow-up, were given a dynamic electrocardiogram every 3-6 months. The average follow-up period was 38.7 +/- 19.8 months, median 36. Sinus node dysfunction was detected during follow-up in 20 patients (33.3% of the total), 8 of whom had had a pacemaker inserted. Risk factors for late development of sinus node dysfunction were found to include prolonged cross-clamping of the aorta during surgery (P = 0.003), especially over 50 minutes (relative risk 3.5:1), and the presence of even transient sinus node dysfunction after surgery (P = 0.006). These observations suggest, first, that sinus node dysfunction may develop after the Mustard operation as a combined effect of extensive atrial surgery and a long period of myocardial ischemia and, second, that the presence of the disease immediately after the operation sometimes indicates that it will recur or persist during follow-up.
Of 353 children who underwent surgical repair of a congenital heart defect, including closure of a ventricular septal defect (VSD), 12 patients (four with tetralogy of Fallot, five with a VSD, and three with a double-outlet right ventricle) developed subaortic stenosis, which was diagnosed one to six years after the surgical procedure. Five patients required surgical treatment of the subaortic stenosis, and one required percutaneous balloon angioplasty. Postsurgical subaortic stenosis appears to be an uncommon progressive acquired disease.
We describe a two-month-old infant with early congestive heart failure due to anomalous origin of the right coronary artery from the pulmonary artery. The diagnosis was made by two-dimensional and color flow Doppler echocardiography, confirmed by angiocardiography, and the case was successfully corrected at surgery. As opposed to the more frequent anomalous origin of the left coronary artery from the pulmonary trunk, this anomaly generally does not cause any typical clinical finding, often becoming an autoptic or surgical surprise after infancy or in adult age.
The pulmonary circulation is dependent on the ductus arteriosus in all patients with pulmonary atresia and intact ventricular septum and in some with pulmonary atresia and ventricular septal defect (tetralogy of Fallot type). To assess the time of ductal closure in these two patient categories, we compared the ages at first operation in 58 patients with pulmonary atresia and intact ventricular septum and 32 with pulmonary atresia and septal defect. The age distribution differed significantly between the groups. Whereas 90% of the children with intact ventricular septum required surgery in the first week of life, 50% of those with ventricular septal defect underwent surgery after the first month and 25% after the third month. The previously described and now confirmed anatomic differences of ductus arteriosus or different levels of endogenous prostaglandins may explain persistent patency of the ductus in pulmonary atresia with ventricular septal defect. The phenomenon may have important clinical implications regarding the timing and choice of surgical procedure.
The bidirectional cavopulmonary anastomosis is a surgical procedure suitable for patients with cyanotic congenital heart disease and univentricular physiology. This operation is able to increase the effective pulmonary blood flow without any additional load on the cardiac work and without any further distortion on the pulmonary artery branches. The cavopulmonary anastomosis can represent the first stage for patients destined for Fontan repair or a definitive palliative operation in high risk Fontan candidates. In order to test the hypothesis of a definitive palliation by cavopulmonary anastomosis in this kind of patients, we evaluated the hemodynamic data before and after this surgical approach and compared these data with their clinical and functional outcome. We evaluated 74 patients submitted to bidirectional cavopulmonary anastomosis by either hemodynamic or functional evaluation. End-diastolic and end-systolic ventricular volumes were significantly reduced by bidirectional cavopulmonary anastomosis (p less than 0.0005). Despite these data and a normal ambulatory ECG, spirometry and echocardiographic analysis, the stress test showed discouraging results. In fact, mean work time and peak heart rate were significantly different from normal values showing an impaired functional capacity of these children. In conclusion we think that bidirectional cavopulmonary anastomosis can not be considered an adequate definitive palliation but it represents a very good stage to preserve the pulmonary arteries and to prepare the systemic ventricle towards the Fontan repair.
Between December 1986 and December 1990, a bidirectional cavopulmonary anastomosis was performed in 27 patients younger than 2 years of age, including 12 with heterotaxia syndrome. Age and weight of patients averaged 14.2 +/- 6.6 months and 8.1 +/- 2.2 kg, respectively. Eleven had pulmonary atresia and 16 had pulmonary stenosis. The main pulmonary artery was ligated in seven patients in the latter group (subsequently reopened in one) and left open in nine (subsequently ligated in two). There were four hospital deaths (15%). All patients were discharged with anticoagulant/antithrombotic therapy to be continued for 6 months. There were two late deaths before further operations (8.7%). Two patients, one with acquired massive pulmonary arteriovenous fistulas and one with progressive common atrioventricular valve regurgitation, subsequently underwent definitive repair (biventricular in one), and both died. Heterotaxia syndrome (p = 0.087) and preoperative mean pulmonary artery pressure higher than 15 mm Hg (p = 0.09) were the only risk factors for overall mortality.
BACKGROUND: Total cavopulmonary connection has been proposed as a rational alternative to atriopulmonary connection for complex Fontan operations. In addition to fluid dynamic advantages proposed by de Leval, total right heart bypass may address an emerging important issue after repair of single ventricle: late atrial arrhythmias. The purpose of this study is to document the postoperative hemodynamic findings in 22 consecutive patients who received a total extracardiac right heart bypass with an inferior vena cava-to-pulmonary artery extracardiac Dacron conduit with a modified Glenn anastomosis (superior vena cava-to-pulmonary artery anastomosis). METHODS AND RESULTS: Twenty-eight patients with complex congenital heart disease underwent this surgical procedure. One patient died (early mortality, 3.5%). Mean follow-up was 13.9 months. Postoperative cardiac catheterization and echo Doppler studies were performed in 22 of the 27 survivors. In 18 of 22 patients, hemodynamic data were satisfactory; a preferential direction of caval flows to both lungs was observed. Echo Doppler assessments show that forward cavopulmonary flow appears as a predominant early diastolic event, in contrast to what occurs in atriopulmonary connections. This hemodynamic model emphasizes the possible role of the diastolic ventricular performance (as a "suction pump") in Fontan circulation. Early postoperative atrial arrhythmias were observed in two of the survivors. CONCLUSIONS: The technical advantages and the hemodynamic benefits of this form of right heart bypass are encouraging. Although the use of artificial material in this procedure is extensive, none of the survivors showed thromboembolic complications or peel formations with narrowing and/or obstruction. Further investigations during a longer follow-up are needed to confirm the early and intermediate results, especially the reduction of late atrial arrhythmias.
We report on two patients with congenitally corrected transposition of the great arteries in situs inversus who underwent successful anatomic repair of associated cardiac anomalies--ventricular septal defect and pulmonary outflow tract obstruction. Surgical intervention was influenced by the recently introduced technique of Ilbawi and colleagues in cases of congenitally corrected transposition in situs solitus. Principles of the correction are as follows: (1) patch redirection of venous flows at the atrial level through an incision in the left-sided right atrium; (2) patch closure of the ventricular septal defect through a right ventriculotomy, baffling the left ventricle to the aorta; and (3) valved conduit interposition between the right ventricle and the pulmonary artery. If the ventricular septal defect is restrictive, it can be safely enlarged by extensive resection of the anterosuperior border, because of the posteroinferior location of the bundle of His in this anomaly. With this technique, the left ventricle and the native mitral and aortic valves are recruited for systemic work, with intrinsic long-term advantages. It is, however, a complex procedure involving substantial use of prosthetic material. More experience is necessary to establish whether this approach is superior to currently available physiologic repairs.
The experience with endomyocardial biopsy in pediatric age is still limited. From February 1986 to August 1990, 144 right ventricle endomyocardial biopsies were performed in 84 patients (age range 33 days--14 years, median age 31 months, weight range 3--57 kgs). Clinical diagnosis was: dilated cardiomyopathy in 50 patients; graft reject in 19; hypertrophic cardiomyopathy in 4; restrictive cardiomyopathy in 5; heart tumor in 3; ventricular arrhythmia in 3. The bioptome was introduced directly, without the use of a long sheath. There were no major complications; 2 patients experienced complete transient atrioventricular block and in 1 case right ventricular perforation occurred. In 11/45 patients (27%) with the clinical diagnosis of dilated cardiomyopathy and available myocardial specimens, acute myocarditis was diagnosed. In 47/65 procedures in the transplanted patients, a moderate to severe rejection was diagnosed. In the remaining patients, endomyocardial biopsy did not help the clinical diagnosis. We conclude that the right ventricular endomyocardial biopsy is a safe procedure in pediatric age; its utility is mostly limited to the diagnosis of acute myocarditis and graft reject after cardiac transplantation.
Among different anatomical causes of subaortic obstruction, anomalous attachment of the mitral valve on ventricular septum is one of the rarest. We report our experience with 4 such cases of subaortic obstruction. Their age at first observation ranged between 1 and 6 months. All patients had viscero-atrial situs solitus, levocardia and normal atrio-ventricular and ventriculo-arterial connections. None had associated cardiac septal defects. Cases with atrio-ventricular canal were excluded. All patients showed a systolic ejection murmur and electrocardiographic features of left ventricular hypertrophy; 3 presented congestive heart failure in the first 3 months of life. Diagnosis was made by 2D-echocardiography and cardiac catheterization in 3 patients and intraoperatively in 1. Only in the last 2 patients, correct diagnosis was made at the initial 2D-echocardiographic examination. Due to left ventricular hypertrophy, in the first 2 patients hypertrophic obstructive cardiomyopathy was originally suspected. One patient died intraoperatively during attempted transaortic resection of subaortic obstruction. Other 2 patients underwent left ventricle-aortic conduit implantation. Of these 2 patients, 1 died 3 months later for sepsis and the other was reoperated upon 3 years later for mitral valve replacement and conduit take-down, with good clinical result. The fourth patient is waiting for surgical intervention. Subaortic obstruction due to anomalous attachment of the mitral valve on ventricular septum may present with early congestive heart failure; the best diagnostic tool is 2D-echocardiography. Left ventricle-aortic conduit may represent a surgical alternative to transaortic resection.
The introduction of new protocols of immune suppression and especially the use of cyclosporine have led to a marked reduction of infective pathology in children receiving transplants. Nevertheless, infections still represent a major factor of morbidity and mortality in these patients. The above study lists the main viral infections, according to apparatus involved, that may arise, also with reference to the time elapsed since transplantation. The most up-to-date diagnostic possibilities for each infection are reviewed together with some indications on therapy which may subsequently be examined in greater detail.
Explore the source record for details and available documents.
Explore the source record for details and available documents.