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PubMed2003

Role of echocardiography versus MRI for the diagnosis of congenital heart disease.

During the last few decades, significant strides have been made in the field of noninvasive imaging for the patient with congenital heart disease. Echocardiography and MRI continue to provide improved means of anatomic and functional assessment in children and adults with congenital heart lesions. This review reports some of the recent advances in tissue Doppler, strain rate, and integrated backscatter, and highlights exciting current and future potential developments in their application. We also discuss advances in MR in evaluation of cardiac anatomy and function in congenital heart disease.

Adult↗
PubMed2004

BMPR2 mutations in pulmonary arterial hypertension with congenital heart disease.

The aim of the present study was to determine if patients with both pulmonary arterial hypertension (PAH), due to pulmonary vascular obstructive disease, and congenital heart defects (CHD), have mutations in the gene encoding bone morphogenetic protein receptor (BMPR)-2. The BMPR2 gene was screened in two cohorts: 40 adults and 66 children with PAH/CHD. CHDs were patent ductus arteriosus, atrial and ventricular septal defects, partial anomalous pulmonary venous return, transposition of the great arteries, atrioventicular canal, and rare lesions with systemic-to-pulmonary shunts. Six novel missense BMPR2 mutations were found in three out of four adults with complete type C atrioventricular canals and in three children. One child had an atrial septal defect and patent ductus arteriosus; one had an atrial septal defect, patent ductus arteriosus and partial anomalous pulmonary venous return; and one had an aortopulmonary window and a ventricular septal defect. Bone morphogenetic protein receptor 2 mutations were found in 6% of a mixed cohort of adults and children with pulmonary arterial hypertension/congenital heart defects. The current findings compliment recent reports in mouse models implicating members of the bone morphogenetic protein/transforming growth factor-beta pathway inducing cardiac anomalies analogous to human atrioventricular canals, septal defects and conotruncal congenital heart defects. The small number of patients studied and the ascertainment bias inherent in selecting for pulmonary arterial hypertension require further investigation.

Adult↗
PubMed1976

Surgical treatment of congenital heart disease with special reference to the application of hypothermia.

This report presents the results of operation for congenital heart disease using two different methods of hypothermia: (1) Immersion hypothermia alone. Of the 782 patients who underwent open-heart operations using this method the results were good in patients whose intracardiac surgical repair took less than one hour (average mortality rate, 5.6%). (2) Rapid extracorporeal cooling. Of the 269 patients with congenital heart diseases such as ventricular septal defect, tetralogy of Fallot, or atrioventricular canal with low cardiac reserve who underwent operation with mild to moderate hypothermia utilizing rapid extracorporeal cooling, the mortality was 11.2%. In the 151 patients with more serious defects, including the extreme form of tetralogy of Fallot, single ventricle, and truncus arteriosus, who underwent open-heart operations with deep hypothermia utilizing extracorporeal cooling, the mortality rate was 15.2%.

Adolescent↗
PubMed2005

A 28-year trend of infective endocarditis associated with congenital heart diseases: a single institute experience.

BACKGROUND: Infective endocarditis (IE) is still one of the major complications of congenital heart disease and, therefore, prevention has always been an important issue. But there has been no large scale investigation of IE in Japan. METHODS: Clinical and microbiological features in 183 patients with congenital heart diseases complicated with infective endocarditis (IE), which were treated in our institute in the last 28 years, were reviewed. RESULT: During the period, the age distribution of the patients shifted to an older age; 80% were older than 15 years in the latest 7 year period. In the underlying diseases more complex conditions increased, such as the post Rastelli operation. Dental or oral diseases were the major preceding events and Streptococcus was the major pathogen throughout the study periods in the data. CONCLUSION: The result indicates the importance of continuing education for the prevention of IE and oral hygiene especially in adult patients with a risk for IE.

Adolescent↗
PubMed2004

The reduction in hospital stay and costs in the care of patients with congenital heart diseases undergoing fast-track cardiac surgery.

OBJECTIVE: To assess the care provided to patients with congenital heart diseases and ischemic heart diseases undergoing cardiac surgery according to the fast-track recovery protocol compared with those undergoing the conventional procedure. METHODS: The transfer of patients from one hospital unit to another was assessed for 175 patients, 107 (61%) men and 68 (39%) women, with ages ranging from 0.3 to 81 years. RESULTS: The discharge rate from the different hospital units per unit of time of the patients with congenital heart diseases treated according to the fast-track recovery protocol compared with that of patients conventionally treated was as follows: a) 11.3 times faster than the discharge rate of patients treated according to the conventional protocol, in regard to the time spent in the operating room; b) 6.3 times faster in regard to the duration of the surgical intervention; c) 6.8 times faster in regard to the duration of anesthesia; d) 1.5 times faster in regard to the duration of perfusion; e) 2.8 times faster in regard to the stay in the postoperative recovery I unit; f) 6.7 times faster in regard to hospital stay (time period between hospital admission and hospital discharge); g) 2.8 times faster in regard to the stay in the preoperative unit; h) 2.1 times faster in regard to the stay in the admission unit after discharge from postoperative recovery; i) associated with reduced costs. The difference was not significant for patients with ischemic heart disease. CONCLUSION: A reduction in the length of hospital stay and costs for the care of patients undergoing cardiac surgery according to the fast-track protocol was observed.

Adolescent↗
PubMed2004

Anaesthesia for children with congenital heart disease undergoing diagnostic and interventional procedures.

PURPOSE OF REVIEW: Anaesthesiologists are often asked to administer sedation and anaesthesia to infants and children with congenital heart disease undergoing diagnostic and interventional procedures. A number of issues are pertinent to a safe and effective provision of care for these patients. The anaesthetic should ensure haemodynamic stability, provide real-life circumstances to ensure diagnostic accuracy, should be easy and reliable to titrate in order to cope with rapidly changing loading conditions and intense but short-lived haemodynamic disturbances. The anaesthetic drugs should not interfere with electrophysiological processes in the cardiac conduction system, and above all anaesthetic recovery should be swift and devoid of side-effects. RECENT FINDINGS: In recent years, new agents have been introduced in the anaesthetic care of these patients. One has studied the haemodynamic and electrophysiological properties of agents such as remifentanil, desflurane, sevoflurane and propofol and their use in children with congenital heart disease. New and older drugs have been combined in balanced techniques to decrease the side-effects associated with monotherapy. Besides these anaesthetic concerns, the controversy about the presence of an anaesthesiologist versus a non-anaesthesiologist still continues. SUMMARY: The newer anaesthetic agents, such as desflurane, remifentanil, sevoflurane and propofol are an integral part of our armamentarium to care for children with congenital heart disease. The combination of these and older drugs allow us to provide anaesthetic care in an effective and efficient way.

Journal Article↗
PubMed2001

Feasibility of electron beam tomography in diagnosis of congenital heart disease: comparison with echocardiography.

OBJECTIVE: To evaluate the feasibility of electron beam tomography (EBT) for morphological assessment of congenital heart disease. MATERIALS AND METHODS: Sixteen subjects were examined by EBT, transthoracic echocardiography, cardiac cine angiography and surgery. EBT scan was performed using single slice continuous volume mode to acquire high-resolution static image. Double dose contrast medium was injected by bolus technique after measuring scan delay time and calculation of proper injection rate. The results of EBT and echocardiography were compared based on the results of cardiac cine angiography and surgical findings. All anomalous components were evaluated in all subjects and grouped according to major cardiac structures. Statistical analysis was performed to compare two modalities' ability to evaluate the anomalies of major structures. RESULTS: EBT was more sensitive to evaluate the anomalies of systemic vessels, pulmonary vessels and small systemic arteries such as coronary artery. EBT was less sensitive to identify the abnormality of cardiac valves such as valvular regurgitation. There was no difference of sensitivity in evaluation of cardiac chambers and septa between the two modalities. CONCLUSION: EBT assisted by transthoracic echocardiography is excellent noninvasive modality to diagnose congenital heart disease.

Adolescent↗
PubMed2001

Normothermic versus hypothermic cardiopulmonary bypass during repair of congenital heart disease.

OBJECTIVE: To compare normothermic cardiopulmonary bypass (CPB) versus hypothermic CPB in pediatric patients undergoing repair of congenital heart disease with focus on biochemical markers for brain damage. DESIGN: Prospective randomized interventional study. SETTING: Postgraduate teaching hospital. PARTICIPANTS: Twenty patients undergoing repair of congenital heart disease. INTERVENTIONS: Patients were randomized to normothermic (36 degrees C) versus hypothermic (25 degrees C) CPB. Serum levels of neuron-specific enolase (NSE) and S-100beta protein were measured in all patients before surgery, immediately after CPB, and 12 and 24 hours after surgery. Blood loss and time for extubation of the trachea were recorded. MEASUREMENTS AND MAIN RESULTS: Before operation, the S-100beta protein and NSE levels were similar in the 2 groups. The S-100beta protein serum level increased significantly after CPB in both groups, whereas no change was found in the NSE level. There was no difference in the change of NSE and S-100beta protein levels between normothermic and hypothermic CPB. Blood loss was significantly less after hypothermic CPB (25 mL/kg/24 h v 42 mL/kg/24 h). Time for extubation was similar. CONCLUSION: No difference was found in the release of brain-specific proteins between normothermic and hypothermic CPB, but blood loss was higher after normothermic CPB.

Cardiopulmonary Bypass↗
PubMedDate not supplied

High association of congenital heart disease with indirect inguinal hernia.

We evaluated 103 patients with indirect inguinal hernia (IIH) for the association of congenital heart disease by echocardiography. Congenital cardiac abnormalities were recognized in 32% of patients with inguinal hernia, which is significantly higher than that reported in a population-based study in Turkey and other population-based studies. Ventricular septal defect and valvular anomalies are the most frequently detected malformations. Our findings suggest that screening for congenital hearth disease is necessary in children with indirect inguinal hernia.

Child, Preschool↗
PubMed2001

Interventional treatment for fetus and newborn infant with congenital heart disease.

BACKGROUND: Catheter intervention has become popular not only in adult patients but in younger patients with congenital heart disease. The early neonatal catheter interventional treatment has also been attempted in conjunction with the development of prenatal diagnosis of the congenital heart disease. METHOD: Recent articles concerning several severe structural cardiac diseases in newborns, such as critical aortic stenosis, complete transposition of the great arteries, premature constriction of the ductus arteriosus, and pulmonary stenosis or atresia with intact ventricular septum are introduced with consideration. RESULTS: This study investigated our own experiences of early neonatal balloon valvuloplasty in a patient with critical aortic stenosis carried out immediately after the delivery following prenatal diagnosis. A case with prenatal diagnosis of premature constriction of ductus arteriosus which could prevent persistent pulmonary hypertension of the newborn by early delivery at 39 weeks and 1 day of gestation were reported. CONCLUSION: Several cardiac interventional treatments performed in the present time during the perinatal period and some prospects in the near future are described in the discussion.

Angioplasty, Balloon↗
PubMed1985

Why does congenital heart disease cause failure to thrive?

Metabolisable energy intake, determined by bomb calorimetry of food, vomit, stool and urine, and resting metabolism, assessed by respiratory gas exchange, were studied in 21 infants with congenital heart disease and nine control infants. Weight for age, growth rates, and daily metabolisable energy intake per kg tended to be lower in infants with heart disease than in control infants. Resting oxygen consumption was high in those infants with pulmonary hypertension and persistent cardiac failure. Energy intake, as a percentage of that recommended for age, correlated with weight gain, and resting oxygen consumption correlated inversely with both percentage body mass index and relative fatness. Failure to thrive in infants with congenital heart disease may be due to a combination of low energy intakes and, in some cases, high energy requirements allowing insufficient energy for normal growth. Increasing the energy intakes of infants with congenital heart disease may be a way of improving their growth.

Body Weight↗
PubMed2000

Risk factors for congenital heart diseases in Alexandria, Egypt.

A matched case control study has been conducted in the children's hospitals in Alexandria, Egypt, during 2 years-period, aiming at investigating the risk factors for the occurrence of congenital heart diseases. Our results showed that the significant risk factors for developing any type of congenital heart disease and ventricular septal defects were: older paternal age at birth, positive consanguinity, positive family history, female sex hormones, irradiation, hazardous maternal occupation, diabetes mellitus and suburban or rural residence. However, some environmental/teratogenic factors were not implicated in the etiology of atrial septal defects or pulmonary stenosis. These findings strongly suggest that environmental factors vary according to the specific type of congenital heart disease. This study emphasizes on the need to instruct the public about the importance of pre-marital counseling and the deleterious effects of various teratogens in the environment.

Case-Control Studies↗
PubMed2005

Preoperative cranial ultrasound findings in infants with major congenital heart disease.

BACKGROUND: Advances in diagnostic testing and surgical techniques have resulted in reduced mortality in neonates with congenital heart disease (CHD) and a major concern for neurological morbidity in the presence of preoperative neurological injury. OBJECTIVES: To determine the incidence and nature of preoperative cerebral ultrasound abnormalities in neonates with major CHD and to examine the relationship between cerebral abnormalities and the type of CHD. METHODS: Retrospective study; inclusion criteria: (1) neonates with major CHD admitted to the NICU over a 3-y period, (2) gestational age >35 wk, (3) documented preoperative cranial ultrasound available; exclusion criteria: (1) small for gestational age, (2) other congenital anomalies and/or chromosomal abnormalities, (3) a 5-min Apgar score <7, (4) congenital infection. Cranial ultrasounds (CUS) were reviewed without knowledge of the cardiac defect. CHDs were categorized. RESULTS: Fifty of 108 neonates with CHD met the inclusion criteria. Twenty-one patients (42%) had abnormalities on CUS. Thirteen of these (26%) had widened ventricular and/or subarachnoid spaces, three (6%) lenticulostriate vasculopathy, one (2%) calcification in the basal nuclei, and four (8%) had acute ischaemic changes. Cerebral abnormalities occurred more frequently in patients with coarctation or hypoplastic left heart syndrome (HLHS) than transposition of the great arteries (TGA) (63% vs 14%; n.s.). CONCLUSION: There is a high incidence of preoperative cerebral ultrasound abnormalities in this group of neonates with major CHD.

Abnormalities, Multiple↗
PubMed2002

Use of an implantable loop recorder in the evaluation of children with congenital heart disease.

BACKGROUND: A recently developed implantable loop recorder (ILR) has been used in adult patients whose syncope remains unexplained in spite of extensive investigations. Syncope in the patient with congenital heart disease presents a diagnostic challenge. We applied this technology to a cohort of pediatric patients. METHODS: We reviewed our experience with an ILR in patients with congenital heart disease with syncope or palpitations after conventional investigations failed to identify a cause for the symptoms. RESULTS: ILRs were implanted in 4 patients with congenital heart disease at 2 centers for investigation of syncope (n = 2), near-syncope (n = 1), and palpitations (n = 1). Implantations were performed at a mean age of 5.9 +/- 0.9 years (4.2 to 7.6 years) and a mean weight of 26.7 +/- 6.6 kg (15.7 to 42.5 kg) with patients under general anesthesia, with no complications. All patients experienced typical symptoms and activated the device appropriately at a median of 86 days (46 to 102) after implantation. Each patient had good-quality data that allowed interpretation of the rhythm. In 2 of 4 cases, a likely cause for the symptoms was identified, with exclusion of more malignant arrhythmic diagnoses in all patients. Escalation of therapy was avoidable in all patients on the basis of the data recorded by the ILR. CONCLUSIONS: Recently developed loop recorder technology can be implanted in the young child without difficulty. The ILR proved to be very useful for excluding malignant arrhythmias as a cause of symptoms in these patients at high risk.

Canada↗
PubMed1994

Energy expenditure in children with congenital heart disease, before and after cardiac surgery.

Failure to thrive is a common feature of children with congenital heart disease. Whether this is the result of poor nutrition or an abnormally high basal metabolic rate is unknown, yet the state of nutrition has a profound effect on the metabolic response to injury and strongly influences the outcome of surgical treatment. The aim of this study was therefore to measure the preoperative and postoperative energy requirements of children with congenital heart disease. Eighteen children (aged 4 to 33 months) were given two oral doses of doubly labeled water (H2(18)O and 2H2O), the first 1 week before operation and the second 6 hours after the end of cardiac surgery. By measuring the relative loss of each isotope from the body water pool, we were able to calculate the rate of carbon dioxide production and therefore total energy expenditure. In five patients, energy expenditure was clearly elevated, suggesting that a raised basal metabolic rate is an important factor in the observed failure to thrive in at least a proportion of such children. Postoperatively, energy expenditure fell to values below normal for healthy children (not having an operation), which suggests that the stress of surgery leads to smaller energy requirements than have previously been thought.

Cardiac Surgical Procedures↗
PubMed2001

Pharmacologic considerations in the neonate with congenital heart disease.

Advances in knowledge about the developing cardiovascular system and compensatory physiologic changes that occur in infants with congenital heart disease have led to new approaches in the management of cardiac failure and arrhythmias. Information about the pharmacologic effects, pharmacokinetics, and pharmacodynamics of newer agents used in the management of congenital heart disease have led to more appropriate use of these medications to prolong survival and improve outcomes.

Adrenergic beta-Antagonists↗
PubMed1984

Diagnosis of complex congenital heart disease: morphologic-anatomic method and terminology.

A summary and brief illustration of the morphologic-anatomic method of diagnosis of congenital heart disease is presented. The principles of scientific neologizing are considered and exemplified. Scientific freedom of speech and expression is commented upon. It is suggested that unnecessary renaming of numerous cardiac anatomic structures and many forms of congenital heart disease be discontinued, and that terminology be de-emphasized.

Heart↗
PubMed2006

[Effects of octreotide acetate on intractable chylothorax after surgery for congenital heart diseases].

We experienced 2 infants in whom octreotide acetate was effective on intractable chylothorax after surgery for congenital heart diseases. They were 8- and 5-month-old. They were diagnosed as having corrected transposition of the great arteries (TGA) and tetralogy of Fallot respectively, and underwent bidirectional Glenn anastomosis and right modified Blalock Taussig shunt. Chylothorax was revealed on the 11th and the 1st postoperative day, and was not improved by any conventional therapy in either case. Then octreotide acetate was infused continuously with 0.1-0.6 micorg/kg/hour for 24 and 7 days. Chylothorax disappeared completely without any complications such as disturbance of blood sugar level or growth retardation. Octreotide acetate was effective and safe even in infants in intractable chylothorax after surgery for congenital heart diseases, as long as used for short period.

Cardiac Surgical Procedures↗