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PubMed2006

Congenital heart diseases of puppies and kittens.

Congenital heart disease (CHD) is defined as a morphologic defect of the heart or associated great vessels present at birth. Abnormalities are caused by alterations or arrests in particular phases of embryonic development of the fetal heart. The term congenital does not imply that the defect was inherited, and the defect may have occurred spontaneously or secondary to a drug or toxin. By studying families of animals with specific CHDs, many defects have also been shown to be heritable.Additionally, if the defect was caused by a spontaneous de novo mutation,that individual has the potential to transmit the mutation to offspring. The diagnosis of CHD is important not only to the health of the patient but to eliminate affected individuals from the breeding pool.

Animals↗
PubMed1987

Advances in surgical management of congenital heart disease in adults.

The spectrum of congenital heart anomalies presenting in adults varies somewhat from that in children. Prolonged exposure to hypoxemia coupled with volume overload or pressure overload can produce myocardial fibrosis and ventricular dysfunction, which complicates surgical correction. Despite these difficulties, surgical correction is possible for most congenital heart lesions in adults, with subsequent improvement in symptoms and survival paralleling that achieved after repair in childhood. In the future, cardiologists and cardiac surgeons will encounter an additional subgroup of adults with congenital heart problems. These are patients who have survived initial correction or palliation of complex heart diseases and later develop complications owing to failure of abnormal native valves or prosthetic valves, narrowing of surgically created conduits, or intrinsic ventricular failure. Such patients will add to the challenge of evaluation and treatment of adults with congenital heart disease.

Adult↗
PubMed1994

Care of the adult with cyanotic congenital heart disease.

In adults with cyanotic congenital heart disease (CHD), medical considerations apply to nonsurgical survivors and to postsurgical patients. The special needs of these groups present a unique challenge for the adult care practitioner. Clinical management revolves around the physiologic consequences of a right-to-left shunt. The care of the cyanotic CHD patient must encompass not only the physiologic variables that set these patients apart from the "typical" adult with acquired cardiovascular disease but also the lifelong psychosocial impact of their congenital heart program.

Adaptation, Physiological↗
PubMedDate not supplied

Prevalence and distribution of children with congenital heart diseases in the central Anatolian region, Turkey.

Congenital heart diseases (CHD) are the most frequent malformation at birth. The aims of this study were to assess the prevalence of congenital heart disease, their different types, and the detection rate among children in the central Anatolian region in Turkey. The study was conducted during an eight-year period (March 1995-December 2002). The prevalence of CHD in a large tertiary care hospital in the central Anatolian region in Turkey was studied. The diagnosis of a structural defect was based on echocardiographic study. The following age groups were considered: neonates, infants and toddlers, preschool children, schoolchildren, and adolescents. In the study period, 1,693 children were found to have CHD; 1253 patients were neonates and infants. Total prevalence of CHD over the study period was 7.77 per 1000 live-born. The prevalence increased from 6.35 to 9.65 per 1000 live births between 1995 and 2002 (p < 0.05). The average age at diagnosis was 2.2 +/- 3.64 years (1 day to 18 years, median 5 months). There were 863 (51%) boys and 830 (49%) girls, with a male/female ratio of 1:1. Isolated ventricular septal defect (32.6%) was the most frequent acyanotic anomaly, and tetralogy of Fallot (5.8%) was the most frequent cyanotic anomaly. The commonest non-cardiac anomalies with CHD were musculoskeletal anomalies. Down syndrome was determined in 83 patients (78.3%) from all syndromic CHD cases. Congenital heart disease is a very significant health problem. It requires urgent measures in terms of organization of early diagnosis and proper management. The prevalence rate is comparable to that of similar developed countries. Increasing incidence of CHD might be attributed to more diagnoses with new technologic development or it may indicate a real increase in the defects.

Adolescent↗
PubMed2001

Prospective study of congenital heart disease in children.

OBJECTIVE: Descriptive analysis of patients with congenital heart disease. DESIGN: Prospective study. SETTING: Professorial Paediatric Unit, Lady Ridgeway Hospital, Colombo 8. METHOD: All patients with congenital heart disease (CHD) admitted to the Professorial Paediatric Unit at Lady Ridgeway Hospital from August 1998 to August 1999 were included in this study. Data were collected using a pretested questionnaire which was filled by one of the authors. Patients were followed up to date. RESULTS: 102 patients, 53 (52.0%) males with congenital heart disease were studied. All patients were evaluated with chest xray, ECG and 2 D echocardiography, except for two. Based on clinical and echocardiographic findings 69 (67.6%) were found to have acyanotic and 33 (32.4%) cyanotic heart disease; 22 (21.5%) had a ventricular septal defect. Tetralogy of Fallot was diagnosed in 10 (9.8%) patients and was the commonest cyanotic CHD. 52 (51.0%) cases were detected between the ages of 2 weeks to 12 months, and 39 (38.2%) were detected routinely at the postnatal examination of the newborn. Only 12 (11.7%) patients underwent corrective surgery, 10 in Sri Lanka, and 2 in India. 19 children died during the study period, a case fatality of 18.6%. CONCLUSIONS: Previous studies have shown that congenital heart disease accounted for between 16% to 30% of deaths in the Professorial Paediatric Unit at LRH. This is the second commonest cause of deaths in the unit. This study has revealed a high case fatality rate among the patients awaiting corrective surgery. Unless surgical care for patients with congenital heart disease in the public sector are improved, many such children will continue to die early due to lack of facilities.

Female↗
PubMed1988

Prenatal detection of congenital heart disease: factors affecting obstetric management and survival.

Our recent experience in the diagnosis and management of fetal congenital heart disease is presented. During an 18-month period from January 1985 to June 1986, 1757 fetal echocardiograms were performed on a total of 989 antenatal patients. Cardiac anomalies were accurately predicted in 74 cases, 34 of which were associated with extracardiac or chromosomal anomalies. Twenty-three pregnancies were electively terminated. Currently the survival rate for ongoing pregnancies is 17%. A false negative diagnosis was made in 16 cases, the majority involving minor anomalies with a good prognosis and a survival rate of 81%. Prenatal detection of congenital heart disease places the fetus at high risk for chromosomal and extracardiac anomalies. Congenital heart disease detectable during pregnancy is usually severe and associated with a poor long-term prognosis. Termination of the pregnancy may be a reasonable option if a severe anomaly is detected early in pregnancy.

Echocardiography↗
PubMed1991

Incidence of congenital heart disease in Tyrol, Austria 1979-1983.

The congenital heart disease incidence study was designed to collect valid data with respect to new diagnostic methods and to an improved system of preventive health care in order to compare the results with former studies. In the years 1979-1983 there were 41,725 live births in the Tyrol. Among these, 341 cases of congenital heart disease were recognised giving an incidence of 8.2 per 1000. This does not imply a real increase in congenital heart disease, but can be explained by differences in methodological bias. As in previous investigations as well in our study, ventricular septal defect has been noted as the most common cardiac lesion. A remarkable result is a 56% incidence of spontaneous closures. Within five years, 81 children died; 89% of them died by the age of one year.

Austria↗
PubMed2001

Health care issues facing adolescents with congenital heart disease.

The number of children with congenital heart disease surviving beyond adolescence is rapidly increasing. Consequently, pediatric health providers not only have to address medical issues associated with the cardiac condition but must begin to develop programs that assist adolescents and their families in dealing with special health care needs for the young patient to successfully move into the adult world. Transitional health-related issues facing the adolescent with congenital heart disease including medical follow-up, insurability, employability, sexuality, and reproduction are described. Discussion about advising and counseling both patient and parents is included.

Adaptation, Psychological↗
PubMed2004

Prevalence of congenital heart disease.

BACKGROUND: Today most patients with congenital heart disease survive childhood to be cared for by adult cardiologists. The number of physicians that should be trained to manage these lesions is unknown because we do not know the number of patients. METHODS: To answer this question, the expected numbers of infants with each major type of congenital heart defect born in each 5-year period since 1940 were estimated from birth rates and incidence. The numbers expected to survive with or without treatment were estimated from data on natural history and the results of treatment. Finally, lesions were categorized as simple, moderate, or complex, based on the amount of expertise in management needed for optimal patient care. RESULTS: From 1940 to 2002, about 1 million patients with simple lesions, and half that number each with moderate and complex lesions, were born in the United States. If all were treated, there would be 750,000 survivors with simple lesions, 400,000 with moderate lesions, and 180,000 with complex lesions; in addition, there would be 3,000,000 subjects alive with bicuspid aortic valves. Without treatment, the survival in each group would be 400,000, 220,000, and 30,000, respectively. The actual numbers surviving will be between these 2 sets of estimates. CONCLUSIONS: Survival of patients with congenital heart disease, treated or untreated, is expected to produce large numbers of adults with congenital disease, and it is likely that many more adult cardiologists will need to be trained to manage moderate and complex congenital lesions.

Adult↗
PubMedDate not supplied

Postoperative chylothorax: differences between patients who received median sternotomy or lateral thoracotomy for congenital heart disease.

BACKGROUND: Chylothorax after surgery for congenital heart disease is rare. We wanted to compare the different presentations of chylothorax in patients who received median sternotomy or lateral thoracotomy. PATIENTS AND METHODS: We retrospectively studied pediatric patients with congenital heart disease who received palliative or corrective surgeries and developed postoperative chylothorax between January 1992 and July 2003. Patients were divided into two groups by the type of surgery: median sternotomy and lateral thoracotomy. The average daily fluid amount (mL/kg/24 hours), latency period, duration of chylothorax, and requirement for surgery were compared. RESULTS: Seventeen patients (11 boys, 6 girls; mean age, 14.0 +/- 12.8-month-old) were enrolled. Diagnoses were tetralogy of Fallot (n = 8), right isomerism with complex heart defects (n = 3), patent ductus arteriosus (n = 2), transposition of the great artery (n = 1), ventricular septal defect (n = 1), and endocardial cushion defect (n = 2). There were 9 in the lateral thoracotomy group and 8 in the median sternotomy group. Only one patient required surgery because of the failure of conservative treatment. The lateral thoracotomy group had a significantly lower average body weight (6.9 +/- 2.9 kg vs. 11.0 +/- 3.8 kg) and longer average latency period before postoperative chylothorax (15.1 +/- 9.2 days vs. 7.2 +/- 4.7 days). CONCLUSION: The majority of pediatric patients who develop chylothorax after cardiac surgery can be successfully managed by medical treatment only. To avoid complications in pediatric patients after cardiac surgery, chylothorax should be suspected for patients with unexplainable, prolonged, and abundant pleural effusion.

Cardiac Surgical Procedures↗
PubMed2005

Saturation with oxygen for ductal dependent congenital heart diseases before and after the prostaglandin therapy.

Ductal dependent congenital heart diseases represent 14-20% of all congenital heart diseases. A primary goal of the treatment of these diseases is to retain ductus open until the final cardiosurgical treatment. Prostaglandins are presently the only medicaments, which have a capability to keep ductus open. By means of a retrospective study in a period from January, 2000 until December, 2002 at the Paediatric clinic of the Clinical centre of the University in Sarajevo, 14 patients (treated with prostaglandins) diagnosed with ductal dependent congenital heart diseases were analyzed. In our sample, there are 9/14 male patients (64.3%), 11/14 (78.6%) were full-term newborns, while 10/14 (71.4%) were eutrophic at birth. An average saturation increase, after the prostaglandin therapy, measured in blood from the capillaries is 29, and measured transcutanlly is 32 units. Duration of prostaglandin therapy in our study was on average 17.2 days. The most common cause of death was insufficientia cardiorespiratoria (4 out of 11), but sepsis/infection (3 out of 11) and insufficientia renalis were also common. 78.6% (11 out of 14) patients died partly because of the complexity of these diseases, but also because a cardiosurgical treatment is delayed. A goal of this study is evaluation of saturation with oxygen before and after the prostaglandin therapy.

Alprostadil↗
PubMed1985

Physician-patient communication. Understanding congenital heart disease.

Mothers' descriptions of their childrens' congenital heart disease were compared with cardiologists' diagnoses. Maternal understanding was examined with respect to 21 factors using bivariate contingency tables and multiple regression analyses. Of 285 mothers, 36% demonstrated poor comprehension. Maternal understanding was associated with sociodemographic variables but not with factors related to stress. All variables taken in concert that were predictive of understanding explained less than 13% of the variability in maternal knowledge. Distorted perceptions of congenital heart disease may cause unnecessary anxieties, inappropriate restrictions, and impairment of the child's self-perception. The bizarre nature of some of the mothers' incorrect descriptions and the accuracy of responses of mothers in biologic and health care occupations suggest that fundamental ignorance of the cardiovascular system may be of such importance as to minimize the impact of other variables.

Attitude to Health↗
PubMed1997

Paradoxical relationship between N-terminal proatrial natriuretic peptide and filling pressure in adults with cyanotic congenital heart disease.

BACKGROUND: Many adults with cyanotic congenital heart disease are characterized by reduced ventricular filling pressures and decreased systemic oxygen transport. Data from animals suggest that hypoxia can induce synthesis and secretion of atrial natriuretic peptide. METHODS AND RESULTS: We measured plasma N-terminal (1-98) proatrial natriuretic peptide (proANP) in 26 cyanotic adults and 28 noncyanotic control subjects. Resting arterial oxygen saturation was significantly lower and hemoglobin concentration and hematocrit significantly greater in cyanotic patients than in control subjects (82+/-6 versus 96+/-3%, 19.7+/-2.2 versus 14.7+/-2.1 g/dL, and 59.0+/-8.5% versus 44.3+/-5.2%, respectively, P<.0001 in all cases). Four cyanotic patients had evidence of iron deficiency. Plasma proANP levels were elevated in cyanotic patients compared with control subjects (1828+/-1147 versus 689+/-343 pmol/L, P<.0001). Comparison of resting arterial oxygen saturation and proANP levels demonstrated an inverse linear relationship between the two measures (r=-.70, P<.0001). There was a significant linear relationship between both hemoglobin concentration and hematocrit and proANP levels as well (r=.53, P=.0003 and r=.48, P=.002, respectively). Cyanotic patients had lower mean right atrial pressures than the control subjects (4+/-3 versus 7+/-2 mm Hg, P=.005), and there were inverse logarithmic relationships between proANP levels and systemic cardiac index (r=-.82, P=.0002), systemic oxygen transport (r=-.68, P=.005), and mixed venous oxygen saturation (r=-.79, P<.0001). CONCLUSIONS: Adults with cyanotic congenital heart disease are characterized by increased levels of plasma proANP. The increased atrial natriuretic peptide most likely results in extracellular and plasma volume depletion and reduced systemic oxygen transport. Measures designed to increase ventricular filling may improve quality of life of these patients.

Adolescent↗
PubMed1996

Family planning requirements in the adult congenital heart disease clinic.

OBJECTIVE: To determine whether women with congenital heart disease were receiving appropriate advice on contraception. SETTING: Adult congenital heart disease clinic in a tertiary cardiac referral centre. DESIGN: Questionnaire administered to 35 consecutive female patients attending the adult congenital clinic. The cardiologist assessed what the risk would be if each patient used an oestrogen containing contraceptive pill (OCP). RESULTS: Of the 33 patients admitted to the study 6 patients thought their heart condition precluded them from taking an OCP when in fact it did not and 3 incorrectly said that an OCP would be suitable for them. Three women with relatively minor lesions had been incorrectly denied the OCP and 2 further patients were using inappropriate methods. There had been 6 unwanted pregnancies in the total group. CONCLUSIONS: Many women with congenital heart disease do not know the most appropriate method of contraception for them or have received incorrect advice. It is often patients with less severe lesions who receive the most inappropriate advice. It is clear that the family planning needs of this population are currently poorly catered for. Each unit must ensure that the information necessary in making informed decisions on contraception is available to the doctor advising on family planning.

Adolescent↗
PubMed2005

Quality of life and specific problems after cardiac surgery in adolescents and adults with congenital heart diseases.

BACKGROUND: Grown-ups with congenital heart disease (GUCH) constitute an increasing population. Some of them reach adulthood without intervention and may present with symptoms, some require first intervention or re-operation for various reasons. Although interventions become more and more frequent in these patients, limited knowledge exists on their quality of life (QoL). The aim of the present study was to analyze QoL in GUCH patients who underwent cardiac surgery after the age of 14 years. DESIGN: A total of 296 patients with a mean age of 35+/-16 years (range 14-72 years) were operated on at our institution between July 1987 and December 2000, mainly for atrial septal defect (ASD), outflow tract lesion, Marfan syndrome and co-arctation. Early mortality was 3.4%. During follow-up QoL was assessed with the short form 36 health survey questionnaire (SF-36) and an additional questionnaire focused on medical and psychosocial aspects. Results were analyzed for the total collective and in relation to the underlying congenital heart disease (CHD). RESULTS: Quality of life was excellent and similar to an age- and gender-matched standard population, except in patients following repair of complete AV-canal. The main restrictions in this group were found in the emotional aspect (62.5+/-29.9) and physical role function (60.5+/-25.0) and reflected in limitations of daily activity. CONCLUSION: Outcome and QoL in adolescents and adults with CHD is excellent and similar to that of an age- and gender-matched standard population. In the future special attention should be focused more strongly on medical follow-up and psychosocial problems in this increasing group of patients.

Adaptation, Psychological↗
PubMed1993

Sonographic diagnosis of congenital heart disease: comparison between the four-chamber view and multiple cardiac views.

OBJECTIVE: To report our experience in the detection of congenital heart disease using both the four-chamber view of the heart as part of the standard obstetric ultrasound examination and multiple cardiac views as part of the detailed targeted examination. METHODS: All admissions to Children's Memorial Hospital of Northwestern University Medical Center with the diagnosis of congenital heart disease between June 1988 and April 1992 were identified (N = 1947). These admissions were matched to deliveries (N = 19,321) that occurred at Prentice Women's Hospital during the same period; of these, 10,004 had at least one obstetric ultrasound examination. All fetuses were scanned either with the standard obstetric ultrasound type of examination, featuring only the four-chamber view of the heart, or by the detailed targeted imaging type of study, featuring multiple cardiac views. The type of examination performed was based on the specific request of the attending obstetrician or gynecologist. RESULTS: Thirty-three neonates who had at least one obstetric ultrasound examination were treated for congenital heart disease. An additional five pregnancies were terminated secondary to serious fetal heart defects. When only the four-chamber view was visualized, 11 of 33 fetuses (33.3%) with confirmed congenital heart disease were detected. CONCLUSIONS: Assessment of the outflow tracts is crucial for detection of many forms of congenital heart disease. However, before this is accepted as the standard of care, both the obstetric and radiologic communities should develop their skills in cardiac imaging. Only then can this sophisticated type of cardiac examination be offered to pregnant women.

Female↗
PubMed2006

Feasibility and clinical impact of live three-dimensional echocardiography in the management of congenital heart disease.

BACKGROUND: Precise assessment of congenital heart lesions requires inferential evaluation from multiple two-dimensional echocardiographic images (2DE). The aim of our study was to assess the usefulness of transthoracic live three-dimensional echocardiography (3DE) in the evaluation of congenital heart disease. METHODS: Eighty-two patients (from 4 months to 31 years, mean age 12 +/- 7.5, 38 males and 44 females), known to have congenital heart lesions, prospectively underwent both 2DE and 3DE. Conventional data acquisition by 2DE and "full volume" 3DE acquisition (apical four chambers, parasternal long and short axes, subcostal windows) were carried out by two independent and blinded operators. Data derived from 3DE were compared to 2DE, and 3DE results were graded into three categories: (A) new findings not seen on 2D echo studies, but not critical to therapeutic decision making; (B) additional anatomic information useful in therapeutic decision making; and (C) information equivalent to 2D echo studies. RESULTS: Two out of 82 patients (2%) were excluded because of suboptimal 3DE images. In comparison with 2DE studies, 3DE was graded A in 23 patients (29%), B in 28 patients (35%), and C in 29 patients (36%). In the patients with group B results, atrial and ventricular septal defects, endocardial cushion defects, and l-transposition of great vessels were the most represented pathologies in which 3DE aided medical or surgical therapeutic options. While the new findings in group A did not influence therapy, they defined the whole spectrum of abnormalities in those patients. In patients who fell under group C results, 3DE provided a direct realistic display of the pathology detected by 2DE. CONCLUSIONS: Our study demonstrates that live 3DE, easily performed at the bedside, provides incremental information on patients with a variety of congenital heart lesions. In the clinical scenario, it clarifies the pathology in all its dimensions, particularly in complex lesions with the incremental information having impact on therapeutic decision making.

Adolescent↗