Complex Cyanotic Congenital Heart Disease Research Articles

Current status of endocarditis prevention

There are currently no randomized and carefully controlled human trials to definitively prove that endocarditis prophylaxis is efficient. Furthermore, most cases of endocarditis are not attributable to a medical procedure. Thus, even with a high level of application of endocarditis prophylaxis only a minority of cases could be prevented. Endocarditis is a rare disease. On the other hand, its morbidity is increasing! In addition, infective endocarditis remains still a major medical concern because of its mortality between 5% and 76%. In addition, in up to 40% of all patients suffering from endocarditis one or more heart valves have to be replaced in the following 5 to 8 years. Without treatment endocarditis has a lethality of 100%. Therefore, there is worldwide agreement that endocarditis prophylaxis is necessary. Combining the recommendations of the German and the American Heart Association, as well as the results of the European consensus conferences, with newer insights into the pathophysiology of endocarditis the following aspects are elucidated: depending on their risk of endocarditis patients are allocated into 3 groups. In the first group there are patients with prosthetic cardiac valves, patients who suffered from previous endocarditis and patients with complex cyanotic congenital heart disease and surgically constructed shunts or conduits of the aorta and/or pulmonary circulation. In these high-risk patients the prophylactic regimen for dental, oral, respiratory tract procedures is oral amoxycillin. In genitourinary and gastrointestinal procedures ampicillin and gentamicin i.v. is recommended. In patients with mostly congenital cardiac malformations, acquired valvular dysfunction, hypertrophic obstructive cardiomyopathy and mitral valve prolapse or thickened leaflets and valvular regurgitation oral amoxycillin is recommended for all medical procedures (second group). The third group consists of patients with isolated secundum atrial defect, previous coronary bypass graft surgery, patients with cardiac pacemakers or defibrillators. In this patient cohort the individual risk of endocarditis is not higher than in the general population. Therefore, endocardits prophylaxis is not recommended.

Cardiovascular disease in pregnancy

Heart disease continues to be an important cause of maternal mortality. This is largely because of the extensive haemodynamic changes that occur during pregnancy, namely the increase in blood volume, increase in cardiac output, fall in systemic vascular resistance and hypercoagulable state. High-risk periods include the end of the second trimester, during labour and the immediate postpartum period. Prognosis depends on the specific heart condition, the patient's functional class and the degree of cardiac dysfunction. Conditions that are contraindications to pregnancy include Eisenmenger's syndrome, primary and secondary pulmonary hypertension, complex cyanotic congenital heart disease, and Marfan's syndrome with aortic root dilatation. Women with heart disease should be thoroughly evaluated and counselled before and during pregnancy. Multidisciplinary care is essential for successful maternal and fetal outcomes.

Pulmonary artery augmentation with autologous aortic tissue.

To assess durability and viability of autologous aortic tissue used to augment severe branch pulmonary artery stenosis with a novice surgical technique. Seven patients underwent corrective surgery for complex cyanotic congenital heart disease. Their age ranged from 3-6 years, and their weight 11-17.4 kg. All had concomitant branch pulmonary artery stenosis repaired utilizing an autologous patch, harvested from the patient's own aorta by excising a ring and opening it to form the patch. The aorta is reconstructed directly by end to end anastomosis. One patient died in hospital. Another patient died at 18 months at home. The surviving five patients have remained well in the follow up period of mean 31 months (range 10-52). All patients were restudied by follow up echocardiography and remain with no evidence of the aortic autograft tissue calcification or stenosis. The reconstructed aorta showed no stenosis at the site of anastomosis. The intermediate term results of this novice surgical technique appear encouraging and justify the technique. However, longer follow up will be required to confirm the continued growth of this patch material.

ANESTHETIC CONSIDERATIONS FOR CHILDREN WITH CONGENITAL HEART DISEASE UNDERGOING NONCARDIAC SURGERY: Answers to Common Questions

ANESTHETIC CONSIDERATIONS FOR CHILDREN WITH CONGENITAL HEART DISEASE UNDERGOING NONCARDIAC SURGERY: Answers to Common Questions

Pulmonary balloon valvuloplasty in the palliation of complex cyanotic congenital heart disease.

To assess the value of pulmonary balloon valvuloplasty in the interim palliation of complex congenital heart disease and pulmonary stenosis in children, who often require numerous palliative operations before definitive surgical repair. Evaluation of pulmonary balloon valvuloplasty procedures performed over a five year period in 18 patients (age 8 days--29 years; mean 5.5 years) with complex cyanotic congenital heart disease. After pulmonary balloon valvuloplasty oxygen saturation increased from a mean (SD) of 69 (7.5)% to 83 (7.0)% (P < 0.001). Mean pulmonary artery pressure increased from a mean (SD) of 11.3 (3.8) mm Hg to 15.7 (3.9) mm Hg (P < 0.001). Transient complete atrioventricular block occurred in one patient. No other complications were encountered. In 5 patients (28%) there was an inadequate improvement in cyanosis compared with pre-procedure values (72 (4.7)% v 66 (8.1)%). Reasons for failure were increasing infundibular stenosis in three and inadequate mixing in one child. In 13 patients (72%) pulmonary balloon valvuloplasty gave adequate interim palliation over a mean follow up of 1.1 (1.3) years. Oxygen saturation was 81 (5.6)% at last follow up compared with 70 (7.3)% before pulmonary balloon valvuloplasty (P < 0.001). Pulmonary balloon valvuloplasty is a safe and effective technique in the palliation of patients with complex cyanotic congenital heart disease associated with pulmonary valve stenosis.

Bidirectional Glenn Shunt for Complex Cyanotic Congenital Heart Disease

The bidirectional Glenn Shunt has been advocated for definitive or staged palliation of patients with a functional single ventricle and decreased pulmonary blood flow who are unsuitable for a Fontan operation. Between April 1990 and July 1994, 86 patients underwent bidirectional Glenn operation at the All India Institute of Medical Sciences in New Delhi. This operation was performed electively in 73 patients (group I), as a take-down for Fontan failure in 10 patients (group II), and to complement a two-ventricle repair in 3 patients in whom the right ventricle or the tricuspid valve was inadequate to handle the entire systemic venous return (group III). Fifty-five other procedures were performed in addition to the cavopulmonary shunt. The mean ICU stay was 2 days. There were 5 early deaths in group I, 6 in group II, and none in group III. Mean oxygen saturation at the time of discharge was 84.2% on room air. Follow-up ranged from 3 to 51 months. The bidirectional Glenn shunt was definitive palliation in 9 patients with azygos or hemiazygos continuation of the inferior vena cava. In the remaining patients, a corrective procedure is planned only if there is evidence of failure of palliation or ideal criteria for the Fontan procedure are met. Only 3 patients from group I have therefore undergone subsequent conversion to a Fontan circulation. The bidirectional Glenn shunt provides excellent relief of cyanosis, does not volume load the heart, and possibly lowers the risk of a subsequent Fontan operation. Timely take-down of a failed Fontan procedure is imperative for successful salvage.

The risk of infective endocarditis after cardiac surgical and interventional procedures.

The risk of infective endocarditis after cardiac surgery relates mainly to the risk of infective endocarditis on prosthetic valves. The frequency of prosthetic infective endocarditis varies according to the criteria used in the literature, ranging from 0.4 to 1.3% for early infective endocarditis, with an annual linear risk of late infective endocarditis of 0.5%. This figure seems to be independent of either the type or the location of prostheses, but it does nevertheless increase if more than one valve has been replaced. The most commonly isolated microorganisms in early infective endocarditis are staphylococci. The bacteriological findings in late prosthetic infective endocarditis are similar to those seen in the native disease. The portal of entry is more easily identified in early than in late infective endocarditis (50%). The risk of infective endocarditis in surgically treated congenital heart disease is very low when the patient has a left-to-right shunt or valvar stenosis; it increases amongst patients with tetralogy of Fallot and patients with complex cyanotic congenital heart disease, mainly when there is a residual ventricular septal defect or prior palliative surgery. The risk of infective endocarditis in patients with intracavitary electrodes such as pacemakers and defibrillators, after the interventional procedure itself and after heart transplant, is very low. This leads us to conclude that antibiotic prophylaxis is only warranted in those patients with a prosthetic valve and after surgical treatment of tetralogy of Fallot and other complex cyanotic congenital heart diseases.

Use of the bidirectional cavopulmonary shunt in patients under one year of age

SummaryBetween January 1990 and March 1992, a bidirectional cavopulmonary shunt was performed in seven patients under one year of age for palliation of complex cyanotic congenital heart disease. Five cases had tricuspid atresia with a restrictive ventricular septal defect. One case presented with complete mirror imagery, right-sided heart, double inlet and double outlet right ventricle with pulmonary stenosis. Another case had isomerism of the left atrial appendages with common atrioventricular valve and double outlet right ventricle. All had a pulmonary arterial index greater than 250 mm/m2(Nakata index). Age and weights before surgery averaged five months (one to 11 months) and 5.6 kg (range 2.8 to 8.5 kg) respectively. Preoperative mean arterial oxygen saturation measured by cardiac catheterization was 60.05±14% and postoperative mean arterial oxygen saturation on room air was 79.85±6.03% (p&lt;0.05). There were no deaths, although postoperative complications occurred in three patients, namely pleural effusion and pneumothorax. Mean follow-up was 10±4.2 months. The cardiothoracic ratio decreased in all cases. On subsequent echocardiographic studies with pulsed Doppler sampling, low velocity systolic-diastolic forward flow was registered in the pulmonary trunk. The velocity of flow increased clearly during inspiration. The bidirectional cavopulmonary shunt can be considered as primary palliation for complex cardiac malformations with restricted pulmonary flow independent of age and body weight.

Quality of life in surgically palliated complex congenital heart disease.

The outcome of surgical palliation was evaluated in 26 children with complex cyanotic congenital heart disease. Outcome was examined in terms of ongoing symptoms, exercise tolerance, and the ability to participate in normal childhood activities. An activity score was calculated and each child performed graded treadmill exercise testing. Breathlessness (24 (92%) children), respiratory infections (nine (35%) children), and leg cramps (eight 31%) children) were the most common physical disorders. Although formal exercise testing showed a clear reduction in exercise tolerance compared with age and sex matched controls, palliation had allowed 23 (89%) to function with moderate exercise limitation, three (11%) having severely limited activity. Parents underestimated the child's exercise tolerance in 80% of cases. Sixteen (62%) patients attended school full time, eight (31%) attended part time, and two (8%) received only home tuition. Palliative surgery can give children with a single functional ventricle a level of activity which allows them to take part in most childhood activities. Subjective estimates of exercise tolerance are inaccurate in this group of children, and formal exercise testing can contribute useful information to decision making about further surgical intervention.

Absence of the arterial duct in a neonate with tricuspid atresia

SummarySummary Absence of the arterial duct has been reported in association with tetralogy of Fallot, with pulmonary stenosis or atresia, other complex cyanotic congenital heart diseases, and anomalies of the aortic arch but not, as far as we are aware, with tricuspid atresia. We report the clinical, surgical, and autopsy findings of a patient with tricuspid atresia whose initial clinical course was influenced by the absence of thearterial duct.

Doppler flow profiles in the right and left pulmonary artery in children with congenital heart disease and a bidirectional cavopulmonary shunt.

The systolic and diastolic Doppler tracings in the right and left pulmonary artery were analyzed in 10 patients with complex cyanotic congenital heart disease, aged 6 months to 12 years (median 3 years), after employment of a bidirectional cavopulmonary shunt. The postoperative interval ranged from 2 weeks to 1.7 years (median 1.3 years). In children with pulmonary atresia or severe pulmonary stenosis with minimal antegrade pulsatile pulmonary blood flow Doppler echocardiography confirmed a systolic and diastolic bidirectional shunt from the vena cava superior to both pulmonary arteries. In children with pulmonary stenosis, Doppler echocardiography confirmed a systolic shunt only to the right pulmonary artery and a diastolic bidirectional shunt into both pulmonary arteries. As the left pulmonary artery was perfused by the pulsatile transvalvular flow it was difficult to detect a concomitant systolic Glenn-related flow in those patients. Quantitative analysis of the diastolic Doppler tracings revealed a significant difference in the velocity time integral in the right and left pulmonary artery indicating a dominant right lung perfusion in diastole.

Detection of intra-atrial conduit leakage by transesophageal echocardiography

A 19-year-old male patient who had undergone total cavo-pulmonary connection for complex congenital cyanotic heart disease with partial anomalous pulmonary venous return, was found to have persistent postoperative cyanosis. Transesophageal echocardiography disclosed leakage of the intra-atrial Gortex conduit with a right-to-left shunt. Prompt re-do operation confirmed the site of the leakage which was successfully repaired. Intraoperative transesophageal echocardiography may be indicated so as to avoid a secondary open-chest procedure for this complication.

Echocardiographic documentation of splenic anatomy in complex congenital heart disease

Splenic abnormalities are associated with complex, cyanotic congenital heart disease (CHD). 1,2 Patients with abnormal spleens may also have an increased susceptibility to infection, 3,4 or additional characteristic gastrointestinal anomalies, 2 For these reasons, it is important to routinely define the splenic status of patients with complex CHD. In the past, determination of splenic status needed either radiation exposure (computed tomography or radionuclide imaging) 5,6 or indirect evidence from blood smears (presence or absence of Howell-Jolly bodies). 1,7 Ultrasonography can image the spleen consistently and clearly. 8 Routine ultrasonic imaging of the spleen as a part of a comprehensive echocardiographic examination has not been reported. This study reports our recent experience with routine visualization of the spleen during complete echocardiographic examinations of patients with complex CHD.

Early and Late Results of the Modified Waterston Shunt with PTFE Grafts for Palliation of Complex Congenital Cyanotic Heart Disease in Neonates

During a 12 year period from 1978 to 1989, 35 infants under 4 weeks of age underwent palliative surgery for complex congenital cyanotic heart disease with a short (1-1.5 cm) PTFE graft between the ascending aorta and the right pulmonary artery (modified Waterston shunt). Twenty-three infants had pulmonary atresia and 14 had severe pulmonary stenosis. Underlying cardiac lesions were tetralogy of Fallot (n = 11), single ventricle (n = 7), transposition complexes (n = 6), and intact ventricular septum and hypoplastic right heart syndrome (n = 13). There were 4 early deaths (10.7%) in the entire series, 2 of which were shunt related. Three of the 4 occurred during our initial experience with this shunt in 1978 and 1979. They led to the modified Waterston shunt being abandoned for 3 years in favor of other shunt procedures. Since 1983 one early death occurred in 28 infants (3.5% mortality) with no death in the latest 26 patients. All patients were followed up between 6 and 108 months. There were 4 late deaths, one of which was shunt related. We observed a significant difference in the shunt patency rate between 4 and 5 mm grafts: palliation was adequate after 2 years in 52% of the patients when a 4 mm graft was used and in 89% of the 5 mm graft group (p less than 0.005). Reshunting was necessary in 7 infants between 5 and 60 months after primary surgery. Recatheterization was performed in 17 infants for suspected shunt failure (n = 6) or diagnostic reasons (n = 11).(ABSTRACT TRUNCATED AT 250 WORDS)

Development of pulmonary arteriovenous malformations after modified Fontan operations

Two patients with complex cyanotic congenital heart disease and without previous Glenn anastomosis underwent modified Fontan operations. Postoperatively, pulmonary arteriovenous malformations developed that resulted in cyanosis from significant intrapulmonary right-to-left shunting. These malformations were detected by pulmonary angiograms and contrast echocardiograms. Pulmonary arteriovenous malformations may be a major cause of late clinical deterioration in patients treated with modified Fontan operations.

Balloon dilatation of stenosed Blalock-Taussig shunts.

Three patients with complex cyanotic congenital heart disease, who had previously undergone Blalock-Taussig shunt operations, developed severe stenoses at the pulmonary anastomosis of the shunts. All were successfully treated with balloon dilatation, which can thus be considered an effective alternative to reoperation.

Bidirectional cavopulmonary shunts: Clinical applications as staged or definitive palliation

A standard Glenn anastomosis between the superior vena cava and the right pulmonary artery has been the accepted mode of treatment for patients with complex cyanotic congenital heart disease. We report our experience in 18 patients with such disease who underwent a bidirectional cavopulmonary shunt because of increasing cyanosis and growth cessation. All patients were considered less than “ideal” candidates for a Fontan procedure. We divided the patients into two groups: group 1 had azygos continuation and group 2 did not. Fourteen patients required hypothermic cardiopulmonary bypass. Bidirectional pulmonary blood flow was achieved in all patients. Only 1 death occurred (group 2). The improvement in oxygen saturation and overall clinical condition of these patients, together with the low mortality and morbidity, is encouraging. However, long-term follow-up is mandatory for a comprehensive evaluation of this surgical approach as definitive palliation or as a first stage for a Fontan operation.

Reversibility of cortical hyperostosis following long-term prostaglandin E1 therapy in infants with ductus-dependent congenital heart disease.

Two neonates with complex cyanotic congenital heart disease, receiving long-term prostaglandin E1 infusion, for 59 and 78 days respectively, demonstrated significant radiographic changes of symmetric cortical hyperostosis of the long bones. Bone biopsies from one of the patients elucidated the histological changes and serial X-rays revealed regression of the hyperostosis. Long-term follow-up after 13 months did not reveal any late bony changes or growth disturbance.

Central aorta-pulmonary artery shunts in neonates with complex cyanotic congenital heart disease

Methods of palliating critical pulmonary oligemia in neonates with complex cyanotic congenital heart disease continue to evolve. Pulmonary artery distortion and other complications of the use of native vessels to increase pulmonary blood flow has led to the more frequent use of polytetrafluoroethylene shunts either in a central position or as a modified Blalock-Taussig shunt. Central aorta-pulmonary artery shunts have largely fallen into disfavor because of previously reported unacceptably high incidences of complications such as shunt thrombosis, congestive heart failure, and pulmonary artery distortion. This report details our experience palliating 23 neonates with pulmonary atresia or severe pulmonary stenosis by placing central aorta-pulmonary artery shunts utilizing a short segment (less than 1 cm) of polytetrafluoroethylene. Although three of the 23 died postoperatively, none of the 23 patients had evidence of shunt thrombosis. Congestive heart failure, a potential complication of any pulmonary artery shunt, was present in eight of the 20 survivors but did not require shunt takedown and was readily controlled by digoxin. Repeat catheterization was performed in 12 patients; pulmonary angiography showed good growth of both pulmonary arteries and there was no evidence of pulmonary artery hypertension. Although minor pulmonary artery distortion was present in two patients, this distortion was centrally located and easily remedied at the time of total correction. Thus we have found the central aorta-pulmonary artery shunt to be an extremely effective and reliable means of palliating pulmonary artery hypoplasia as a result of pulmonary atresia or severe pulmonary stenosis in neonates.

A method of treating serous fluid leak from a polytetrafluoroethylene Blalock-Taussig shunt

A 3 1/2-year-old girl with complex cyanotic congenital heart disease underwent palliation with a modified Blalock-Taussig shunt, for which a 6 mm polytetrafluoroethylene graft was used. Seroma formation with serous leakage occurred. Two periods of conservative management failed. At reoperation, treatment of the graft with intraluminal "fibrin glue" resulted in immediate resolution of the leak.