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Anatomic repair of corrected transposition or atrio-ventricular discordance: report of 8 cases.

BACKGROUND: It is well recognized that a right ventricle (RV) may eventually fail if working as systemic. This has led to arterial switch operation for transposition of the great arteries (TGA), but more recently to anatomic correction of 'corrected transposition' or atrio-ventricular discordance (AVD) combining an atrial switch and a ventricular outflow repair (arterial switch or Rastelli type). METHODS: We report here 8 cases of such anatomic correction of AVD in patients from 3 months to 10 years. Of these patients, 6 had situs solitus, and 2 situs inversus; 7 patients had a ventricular septal defect (VSD); 5 had an increased pulmonary flow, and 3 had a decreased pulmonary flow with pulmonary stenosis (PS) or pulmonary atresia (PA). In all, 6 had discordant and 1 had concordant ventriculo-arterial (VA) relations, and 1 had double-outlet RV, 2 had an associated coarctation, and 1 had a cor triatriatum. Of the 8 patients, 5 had pulmonary artery hypertension, 3 a moderate to severe tricuspid insufficiency and 6 had undergone one or more previous operations. Correction was done between 3 months and 10 years of age, with 7 having a Senning procedure (twice left-sided) and 1 a Mustard type operation. VA outflows were repaired with: 4 arterial switches, 1 left ventricle to aorta rerouting, 1 right ventricular outflow tract (RVOT) repair and 2 Rastelli. A tricuspid annuloplasty was done once. RESULTS: All patients survived the operation, but 2 patients needed a reoperation (both successful): late obstruction of pulmonary venous channel after a left-sided Senning, and residual VSD closure. Mean follow-up is 45 months (3-122). Of the 8 patients, 6 patients are in sinus rhythm, 2 have junctional rhythm. A patient with junctional rhythm and occasional tachycardia died suddenly 18 months after surgery 1 week after satisfactory evaluation. All have a normal left ventricle function as evidenced by echocardiography. CONCLUSIONS: Despite a more demanding procedure, the 'anatomic repair' of hearts with AVD is possible, even in infancy, with good early and mid-term result. Despite the potential late rhythmic problems of the Senning operation (somehow more frequent with AVD in our experience), it may become, with increasing experience, the procedure of choice in corrected transposition.

Coronary Angiography↗

Application of inverted J-shaped partial sternotomy in intracardiac operations.

OBJECTIVE: To report our experience of intracardiac surgery with an inverted J-shaped partial sternotomy (IJPS) and describe the technical details of this approach. METHODS: From January to December 2002, 31 patients underwent intracardiac operations using the IJPS instead of a standard conventional full-length median sternotomy (FLMS). Twenty-one patients had mitral valve replacements (MVR), with combined tricuspid valvuloplasty in three, six had closure of secundum atrial septal defects, three had closure of ventricular septal defects and one had repair of cor triatriatum. Data from 26 randomly selected patients who had undergone MVR through a conventional FLMS were included for comparison. RESULTS: There were no perioperative or in-hospital deaths in either group. There was a significant difference between the two MVR groups in incision length, mediastinal drainage and blood transfusion volume, but not in cardiopulmonary bypass time, aortic cross-clamp time, duration of operation and duration of ventilatory support. There were no conversions from IJPS to FLMS, indicating that access was satisfactory for all the procedures. All patients were discharged without any significant postoperative complications. CONCLUSION: The IJPS is aesthetically pleasing with regard to cosmesis and does not affect safety and exposure. Furthermore, the smaller incision resulted in a significant reduction in blood loss and blood requirements.

Cardiac Surgical Procedures↗

The correction of congenital heart defects with less invasive approaches.

BACKGROUND: The minimally and less invasive approaches for the surgical treatment of different heart diseases are rapidly increasing because of their cosmetic and recovery-related advantages. Presented here are the results of less invasive surgery in 51 patients with congenital heart defects. METHODS: From June 1996 to January 1999, we used less invasive techniques for the correction of congenital heart defects in 51 patients. In 32 patients, we performed right anterolateral thoracotomy (6-13 cm), and on the other 19 patients, we used the partial inferior sternotomy (4-7 cm). The ascending aorta and the caval veins were cannulated in all patients. RESULTS: The following congenital heart defects were corrected: ostium secundum atrial septal defect (n = 35), sinus venosus atrial septal defect with partial anomalous pulmonary venous connection (n = 7), ventricular septal defect (n = 7), tetralogy of Fallot (n = 1), and cor triatriatum sinistrum (n = 1). The average age of the patients was 15 years old ranging from 2 months to 48 years and the average weight 39.6 kg (range 3.8-86 kg). The patients were removed from artificial respiratory support on average 8 hours (range 1-48 hours) after surgery and left the hospital after 7 days (range 2-10 days). In 16 patients, blood transfusions were required, an average 5.7 ml/kg BW (range 1.45-19.75 ml/kg BW). The postoperative course was uneventful in all patients. Follow-up (range 3-33 months, mean 17.5 months) was complete with no late deaths or residual defects. CONCLUSION: The right anterolateral thoracotomy and the partial inferior sternotomy provide a safe approach for the correction of certain congenital heart defects. These techniques enable operative correction without any additional risks being incurred and can be performed with standard instruments and cannulation. Additional approaches for extracorporeal circulation are unnecessary.

Adolescent↗

Correction of congenital heart defects in Jehovah's Witness children.

UNLABELLED: Between August 1989 and July 2003 14 Jehovah's Witness children with congenital heart defects (CHD) aged under 14 years (median 2.9 years) and with a median weight of 14 kg underwent 16 operations with cardiopulmonary bypass (CPB). Five children had been operated on previously between one to three times. Preoperatively, 7 children were prepared with oral iron supplementation and 10 received erythropoietin. Mean hemoglobin (Hb) at admission was 14.4 g/dl (range 10.9 - 19.2). The cardiopulmonary bypass (CPB) circuit was modified to reduce total priming volume. High doses of aprotinin were administered. The modified ultrafiltration (MUF) circuit, used in 7 patients, was parallel to the ECC circuit with continuous circulation of the blood through a small shunt between the arterial and venous lines. Operations performed consisted of VSD closure (3 pts.), ASD closure (3 pts.), Fontan operation (2 pts.), and complete AV canal correction, aortic commissurotomy, Ross operation, Glenn shunt, cor triatriatum correction, MV reconstruction combined with left outflow tract stenosis resection, correction of absent pulmonary valve syndrome, and correction of tetralogy of Fallot in one patient each. There were no deaths. Mean duration of CPB was 192 min and mean aortic cross-clamp time 40 min. The Hb value at the end of the operation was 4.9 - 14.5 g/dl (mean 9.6) and at discharge it was 7.1 - 14.5 g/dl (mean 15.5). No blood or blood products were used in any patient. CONCLUSION: Bloodless cardiac surgery with and without CPB can be safely performed in Jehovah's Witness infants and children.

Aprotinin↗

[Two-dimensional transesophageal echocardiography: a better presentation of intra-atrial structures].

In seven patients with suspected or detected abnormal intra-atrial structures on 2-dimensional echocardiography, transoesophageal echocardiography was diagnostic of left atrial myxoma in two, left atrial thrombus in two, cor triatriatum in two and right atrial membrane in one. The transoesophageal technique extends diagnostic possibilities. The accurate demonstration of atrial abnormalities in such cases by the transoesophageal technique obviates the need of invasive pre-operative angiographic and haemodynamic procedures.

Adult↗

Transoesophageal echocardiographic evaluation of atrial morphology in children with congenital heart disease.

In a prospective study, atrial morphology was evaluated by both transoesophageal and precordial echocardiography in 86 unoperated children with congenital heart disease (age range = 0.2 to 14.8 years, mean = 3.8 years) to determine what advantages, if any, might be inherent in the transoesophageal approach. The information derived from both ultrasound approaches was correlated and compared to information obtained during subsequent cardiac catheterization (78 patients) and, or, surgical inspection (53 patients). Atrial appendage morphology and hence atrial situs was determined by transoesophageal echocardiography in every case (82 solitus, two right atrial isomerism, two left atrial isomerism). In addition, the transoesophageal approach indicated left juxtaposition in four patients, compared to only one by precordial examination. Probe patency of the foramen ovale was correctly predicted in 21 patients by transoesophageal imaging, but in only 10 by precordial imaging. In two children significant secundum defects, undetected by the precordial route, were identified. Multiple atrial septal defects were correctly defined in four patients by transoesophageal study but in only one by precordial study. Sinus venosus defects were documented in four by the transoesophageal approach, but in only one by the precordial. Primum defects were equally well documented (nine patients) by either technique, but the associated valve leaflet morphology was better documented by transoesophageal study in 5/9. A subtotal cor triatriatum was diagnosed in one child only by transoesophageal investigation. Transoesophageal echocardiography allows a much more detailed evaluation of atrial morphology than precordial imaging even in infants. It provides direct diagnosis of atrial situs, detection of juxtaposed atrial appendages and improved demonstration or definitive exclusion of atrial septal defects.

Adolescent↗

Scanning suprasternal echocardiography.

Scanning suprasternal echocardiography was performed in 280 patients with a variety of cardiac anomalies. By using the special suprasternal transducer on the suprasternal notch, the aortic arch, right pulmonary artery, and left atrium were recorded superoinferiorly. From this reference position various scanning techniques were made to record the main pulmonary artery, pulmonary valve, ascending aorta, aortic valve, and distal aortic arch, wherever possible. These scans made it possible to assess (a) the integrity and size of right pulmonary artery and main pulmonary artery in pulmonary atresia, stenosis of origin of right pulmonary artery, pulmonary artery banding, kinked Waterston anastomosis, and aneurysm of pulmonary artery; (b) relative positions of aortic valve and pulmonary valve in malposition complexes; (c) the position and size of the aortic arch in tetralogy of Fallot and aortic coarctation. Measurements of the left atrium made by suprasternal echocardiography were consistently larger than those made by praecordial echocardiography. Changes in relative sizes of aortic arch, right pulmonary artery, main pulmonary artery, and left atrium were also documented in the various cardiac anomalies. The atrial baffle after Mustard repair for d-transposition of the great arteries and the atrial membrane in cor triatriatum were also demonstrated. The introduction of scanning techniques has thus widened the scope of suprasternal echocardiography.

Adolescent↗

Remnant of the common pulmonary vein mistaken for a left atrial mass: clarification by transoesophageal echocardiography.

Faulty incorporation of the common pulmonary vein leaves it as a distinct structure posteriorly, into which the pulmonary veins empty. This "chamber" is separated from the anterior "fetal" left atrium (containing the left atrial appendage and communicating with the mitral valve) by a diaphragm, and is known as cor triatriatum, one of the rarest of cardiac malformations. Less pronounced but still incomplete regression of this vein would result in the persistence of a portion of the common pulmonary vein appearing as a mass along the lateral wall of the left atrium at the junction of the left atrial appendage and left upper pulmonary vein. In two patients, both referred for evaluation of a left atrial mass, transoesophageal echocardiography identified the "mass" as a remnant of the common pulmonary vein. Cardiologists need to be aware of this structural remnant and its possible variants so as to avoid misdiagnosis of this prominence as an atrial tumour or mass.

Aged↗

Delayed recognition of congenital heart disease.

OBJECTIVE: The aim of this prospective study was to evaluate the proportion of children with delayed recognition of congenital heart disease (CHD). METHODS: Of the 744 children with CHD primarily diagnosed during a 10 year period in one hospital, the patients were identified where the diagnosis of CHD was established with a significant delay. RESULTS: Sixty six patients (8.9%) had delayed diagnosis of CHD. Among patients with cyanotic CHD, 10.4% (7 of 67 cases) were referred after they had initially been discharged home from the birth clinic. Among patients with acyanotic CHD, 8.7% (59 of 677) of all children and 35.1% (59 of 168) of the children who required surgery or interventional catheterisation were referred at an age where elective repair should have already been performed or needed immediate treatment because of their haemodynamic status. Of the 66 patients with delayed diagnosis, one infant with cor triatriatum died at admission because of delayed referral and 10 children had severe complications: preoperative cardiogenic shock in seven cases of aortic coarctation and one case of endocardial fibroelastosis, pulmonary hypertensive crisis in one child after delayed repair of a ventricular septal defect, and infectious endocarditis after dental care in a teenager with undiagnosed moderate aortic stenosis, who required Ross operation a few months later. DISCUSSION AND CONCLUSION: A substantial proportion of CHD was detected with relevant delay. In all cases of late diagnosis, clinical cardiac findings were present that should have alerted the physician on the possible presence of underlying CHD.

Adolescent↗

Abnormalities of the pulmonary veins: evaluation with MR imaging and comparison with cardiac angiography and echocardiography.

Seventy-seven patients underwent T1-weighted spin-echo magnetic resonance (MR) imaging. Group 1 (n = 56) consisted of patients with various types of congenital heart disease but normal pulmonary veins. Group 2 (n = 22) consisted of patients with the following conditions: partial anomalous pulmonary venous connection (n = 11), total anomalous pulmonary venous connection (n = 5), cor triatriatum (n = 4), or pulmonary vein stenosis (n = 2). In group 1, the sites of connections of all four pulmonary veins were identified with MR imaging in 88% of cases; the connections of at least three pulmonary veins were seen in all patients. In group 2, the prospective detection rate of pulmonary venous abnormalities with MR imaging was 95%. The prospective detection rates of pulmonary venous abnormalities with cardiac angiography (n = 13) and echo-cardiography (n = 13) were 69% and 38%, respectively. This study indicates that MR imaging can accurately demonstrate the normal pulmonary veins and abnormalities of the pulmonary veins.

Adolescent↗

Echocardiographic features of congenital left ventricular inflow obstruction.

The echocardiographic features of congenital left ventricular inflow obstruction are described in six patients. The echocardiograms in two patients with cor triatriatum were distinguished by normal mitral valve motion and an abnormal echo within the left atrium. In two patients with supravalvar mitral ring, in addition to abnormal mitral valve motion, an abnormal echo, presumably originating from the obstructive membrane, was located between the anterior and posterior mitral leaflets. In two cases of parachute mitral valve, mitral valve motion was abnormal. In one of these cases there were multiple mitral valve echoes similar to those found in supravalvar mitral ring. The echocardiographic identification of an obstructive membrane within the left atrium is difficult because of the occurrence of artifacts. However, membranes may be identified if careful scanning techniques are employed in patients in whom left ventricular inflow obstruction is suspected. The echocardiogram is useful in detecting mitral valve abnormalities in these patients and is valuable in cases where mitral valve replacement is contemplated.

Adolescent↗

Apex echocardiography. A two-dimensional technique for evaluating congenital heart disease.

We have evaluated apex echocardiography, using an 80 degrees phased array sector scanner, in 368 patients with congenital heart disease. With the patient lying with the left side dependent, the transducer is placed over the apex of the heart and cross sectional images are obtained in the plane perpendicular to the cardiac septa and through the orifices of the mitral and tricuspid valves. In this view, the chambers are side by side and both atria and ventricles are separated by their respective septa and atrioventricular valves. Defects in the region of the septa can be detected. Congenital defects involving the atrioventricular valves, such as endocardial cushion defects, tricuspid atresia, and Ebstein's anomaly, can be defined. The location of the baffle after Mustard's operation for aortopulmonary transposition and intra-atrial structures, such as the membrance in cor triatriatum, can be seen. The position of the apex of the heart can be located in dextro, levo, or mesocardia by definition of the apex image. The relative size of the ventricular septum can be identified with the apex image. We have found this technique to be valuable in patients with congenital heart disease who are undergoing cross sectional echocardiography.

Adolescent↗

Congenital left ventricular inflow obstruction evaluated by two-dimensional echocardiography.

Several forms of congenital heart disease that cause left ventricular inflow obstruction have similar M-mode findings, and frequently the exact anatomic diagnosis cannot be made by M-mode echocardiography alone. We examined five children with various forms of left ventricular inflow obstruction using two-dimensional echocardiography. The diagnosis was confirmed by cardiac catheterization and surgery in all five patients. In one patient with congenital mitral valve stenosis, a thick mitral valve with two papillary muscles was imaged. This patient was easily distinguished from a second child with parachute deformity of the mitral valve in whom a single papillary muscle arising from the left ventricular apex was seen. These two patients with mitral valve stenosis were easily differentiated from the three patients in whom the left ventricular inflow obstruction was caused by a membrane within the left atrium. The membrane could be seen in several spatial planes; however, we could not distinguish by two-dimensional echocardiography one child who had cor triatriatum from the other two patients who had a supravalvar mitral ring. Because of its spatial anatomic display, the two-dimensional echocardiogram provides information for a more detailed anatomic diagnosis in children with congenital left ventricular inflow obstruction.

Adolescent↗

Persistent left superior vena cava and associated structural and functional considerations.

Potentially significant associations are presented between anomalous systemic venous return (including both left superior vena cava and left hepatic venous drainage to the coronary sinus), a history of atrial fibrillation and a forme fruste of cor triatriatum in an elderly woman. Lack of associated structural defects or functional deficits makes it difficult to assess the frequency of occurrence in the general population of bilateral superior venae cavae in association with a persistent left hepatic vein draining into the coronary sinus. However, the potential for these systemic venous anomalies needs to be considered when unexplained arrhythmias are encountered. More specifically, the size of the coronary sinus needs to be assessed in patients with arrhythmia.

Aged↗

Mitral clefts and interatrial septum defects: 15-year results.

Between 1985 and 2002, 60 patients (58% female) with a mean age of 20.3 +/- 12.1 years (range, 2-55 years) were treated for anterior mitral leaflet cleft. There was a primum atrial septal defect in 52 patients (87%) and a secundum type in 8 (13%). Concomitant cardiac defects were patent foramen ovale in 6 patients, cleft tricuspid valve in 3, ventricular septal defect in 2, cor triatriatum in 1, and persistent left superior vena cava in 1. Mean grade (1-4) of mitral insufficiency was 2.28 +/- 0.74. Atrial septal defects were closed with a pericardial patch in 45 patients, with a prosthetic patch in 11, and primarily in 4. Mitral leaflet clefts were repaired using interrupted sutures. There was no early or late mortality. Two patients (3%) needed a permanent pacemaker. Postoperatively, severe (> or =grade 3) mitral insufficiency developed in 2 patients; valve replacement was performed in one, cleft recurrence and leakage from the patch were treated in the other. Freedom from reoperation was 92.2% +/- 5.6% at 15 years. Surgical intervention can be performed for congenital anterior mitral leaflet cleft and interatrial septal defect with good results in both pediatric and adult age groups.

Adolescent↗

Cardiac disease in the wheezing child.

Cardiac and pulmonary disease are so closely interrelated that it is often difficult to determine in young infants which is the primary offender. As illustrated in these case reports, failure to recognize the true nature of the disease process may lead to unnecessary procedures and delays which can be life-threatening. Statistically, the wheezing infant very likely is suffering from primary pulmonary disease; however, congenital cardiac abnormalities can cause pulmonary symptoms which completely dominate the clinical picture and lead to erroneous diagnoses. Although rare, the basis of cough and wheezing may be a vascular ring which encircles and compresses the trachea. Lesions associated with large left-to-right shunts, such as ventricular septal defect and patent ductus arteriosus, also can cause cough and wheezing because of bronchial compression by a large tense pulmonary artery and a distended left atrium. These same lesions also produce isolated left ventricular failure with pulmonary venous congestion and episodes of cough and wheezing. Anomalous pulmonary venour return, cor triatriatum, supravalvular mitral ring, or mitral stenosis are other lesions which can cause cough and wheezing secondary to pulmonary venous obstruction.

Bronchial Diseases↗

Left-to-right shunt via left superior vena cava communication with left atrium.

The findings in a 22-year-old man with Ebstein's anomaly of the tricuspid valve, Wolff-Parkinson-White syndrome, and a persistent left superior vena cava are reported. This is the first reported case of this combination of anomalies in which the atrial septum was intact and the left superior vena cava communicated with the left atrium. Uniquely, blood was shunted left to right via the left superior vena cava from the left atrium. Only one previous case of left-to-right shunting via a left superior vena cava (in the absence of mitral valvular disease or cor triatriatum) has been reported (associated with aortic coarctation). Angiograms demonstrated the left atrial connection of the left superior vena cava to be at the entrance of the right superior pulmonary vein into the left atrium. In the absence of demonstrable left-sided heart disease, this anatomic juxtaposition is suggested as a possible explanation for the direction of shunting.

Adult↗

Preterm infants with congenital heart disease and bronchopulmonary dysplasia: postoperative course and outcome after cardiac surgery.

OBJECTIVE: Success in treatment of premature infants has resulted in increased numbers of neonates who have bronchopulmonary dysplasia (BPD) and require surgical palliation or repair of congenital heart disease (CHD). We sought to investigate the impact of BPD on children with CHD after heart surgery. METHODS: This was a retrospective, multicenter study of patients who had BPD, defined as being oxygen dependent at 28 days of age with radiographic changes, and CHD and had cardiac surgery (excluding arterial duct ligation) between January 1991 and January 2002. Forty-three infants underwent a total of 52 cardiac operations. The median gestational age at birth was 28 weeks (range: 23-35 weeks), birth weight was 1460 g (range: 431-2500 g), and age at surgery was 2.7 months (range: 1.0-11.6 months). Diagnoses included left-to-right shunts (n = 15), conotruncal abnormalities (n = 13), arch obstruction (n = 6), univentricular hearts (n = 4), semilunar valve obstruction (n = 3), Shone syndrome (n = 1), and cor triatriatum (n = 1). RESULTS: Thirty-day survival was 84% with 6 early and 6 late postoperative deaths. Survival to hospital discharge was 68%. There was 50% mortality for patients with univentricular hearts and severe BPD. The median duration of preoperative ventilation was 76 days (range: 2-244 days) and of postoperative ventilation was 15 days (range: 1-141 days). The median duration of cardiac ICU stay was 7.5 days (range: 1-30 days) and of hospital stay was 115 days (range: 35-475 days). Current pulmonary status includes on room air (n = 14), O2 at home (n = 4), and ventilated at home (n = 4) or in hospital (n = 4), and 5 patients were lost to follow-up. CONCLUSIONS: BPD has significant implications for children who have CHD and undergo cardiac surgery, leading to prolonged ICU and hospital stays, although most survivors are not O2 dependent. Postoperative mortality was highest among patients with univentricular hearts and severe BPD. Optimal timing of surgery and strategies to improve outcome remains to be delineated.

Bronchopulmonary Dysplasia↗