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PubMed2004

Growth and development of children with congenital heart disease.

BACKGROUND: Children with congenital heart disease (CHD) commonly experience delayed growth. Because growth and development are closely related, both should be considered when a child's progress is examined. PURPOSE: This paper reports a study to evaluate and compare the growth and development of preschool children with CHD to those of normal preschool children. METHODS: The heights and weights of 42 preschool children with CHD and 116 normal preschool children were compared with standard growth curves. Differences in development of personal and social skills, fine motor skills and adaptability, language, and gross motor skills were evaluated. Developmental skills were assessed using the Denver Developmental Screening Test II. RESULTS: A significant difference was found in both body height (P < 0.05) and weight (P < 0.05) between the two groups. More preschoolers with congenital hear disease were below the 50th percentile in height (P < 0.05) and weight (P < 0.001). Preschoolers with CHD had more suspicious interpretations than non-CHD preschoolers, specifically in the language (P < 0.01) and gross motor sections (P < 0.001). Nevertheless, there were two items in the personal-social section and one in the language section on which the children with heart disease passed in the range of 55.6-63.2%. Problems were encountered with the Denver II test because of differences in language, culture and childrearing methods between Taiwanese and Western societies. These cultural differences must be considered when the test is used to assess development. CONCLUSIONS: Learning about the growth and developmental differences between children with CHD and normal children may help parents of the former to detect problems associated with delayed growth and development earlier. These children and their families should have the opportunity to participate in a long-term, follow-up programme that provides information and encourages developmental progress. The results could serve as a reference for those in both clinical and community workers who provide nursing care to children with CHD.

Child↗
PubMed1992

Noncardiac surgery in the patient with congenital heart disease.

The patient with congenital heart disease who presents for noncardiac surgery requires careful evaluation and planning to avoid adverse perioperative events. This chapter presents a physiological approach to the management of anesthesia for the most common congenital heart lesions. The various congenital heart defects are categorized into lesions resulting in: (1) left-to-right shunting; (2) right-to-left shunting; (3) complete mixing of pulmonary and systemic circulation; (4) complete separation of the pulmonary and systemic circulations; (5) increased myocardial work; and (6) mechanical obstruction of the airway.

Anesthesia↗
PubMed1981

Choanal atresia and congenital heart disease.

The association of congenital heart disease with choanal atresia is described in 6 infants (2 White, 3 Black and 1 Coloured). The literature is reviewed and 46 similar cases are analysed. The combination of choanal atresia and congenital heart abnormality generally carries a poor prognosis.

Female↗
PubMed1996

[The influential factors of formation and development of personality in children with congenital heart disease].

106 children with congenital heart disease (CHD) were tested by the Eysenck personality Questionaire (EPQ) and the Psychological Monitor Card in this study. The result showed that the personality of the CHD children is more introversive and moody than that of normal children. The influential factors including disease, family, and society were analysed. It suggested that it is important to provide psychological support to the CHD children and psychological knowledge to the parents.

Child↗
PubMedDate not supplied

Growing up heartsick: the experiences of young women with congenital heart disease.

Young women with congenital heart disease (CHD) now survive to confront issues of sexuality, contraception, and pregnancy. Researchers have examined reproductive abilities and infant outcomes in these women, but have not addressed quality-of-life issues. Grounded-theory techniques were used to interview 13 women about the experience of growing up with CHD. The core variable identified was "growing up heartsick." This variable included manifestations of growing up with heart disease, such as feeling different from others, parental overprotectiveness, and fears of death. Two other related variables were also found: "growing up female" and "living against the body." "Growing up female" was characterized by concerns about fertility, contraception, and pregnancy in relation to CHD. Many of the participants were uninformed about their heart disease and reproductive matters. "Living against the body" was expressed in the women's feelings about their body size and their surgical scars. There were overlapping aspects of the categories; for example, manifestations of "growing up heartsick" appeared in each of the other categories.

Adaptation, Psychological↗
PubMed2000

Noninvasive imaging in congenital heart disease.

Imaging algorithms in congenital heart disease, as in the patient with acquired heart diseases continue to evolve, with more and more information gleaned noninvasively. The emphasis will be on the newer aspects of imaging, not cross sectional echocardiography with color Doppler.

Adrenergic beta-Agonists↗
PubMed1990

Infantile lobar emphysema in association with congenital heart disease.

Three cases of congenital heart disease (CHD) are presented; all infants developed the unusual complication of lobar emphysema (LE). In all cases delay in reaching the correct diagnosis resulted in two of the infants undergoing a second operation to resect the emphysematous lobe. The chest radiograph in all cases was dominated by the heart disease and the radiological signs of LE were initially missed. The radionuclide ventilation/perfusion lung scan was used in two of these infants to provide conclusive proof and to emphasise the clinical significance of the condition. Lobar emphysema in association with CHD should be recognised early and lobectomy performed at the same time as surgical correction of the heart defect.

Female↗
PubMed2002

Approach to congenital heart disease in the neonate.

Congenital heart disease in the newborn is a unique and complex problem faced by both pediatricians and cardiologists as it requires skillful handling and balancing of both neonatal issues as well as cardiac physiology. Babies with cyanotic and acyanotic heart disease are physiologically different from each other and management has to be tailored to the individual diagnosis and clinical status. Rapid diagnosis and appropriate management is the key to reducing mortality and morbidity in this fragile patient population. The following review deals with the common presentations of heart problems in a newborn baby and methods of diagnosis, investigative modalities, recent advances and approach to management.

Diagnostic Imaging↗
PubMed1999

Caring for infants with congenital heart disease and their families.

Congenital heart defects are classified into two broad categories: acyanotic and cyanotic lesions. The most common acyanotic lesions are ventricular septal defect, atrial septal defect, atrioventricular canal, pulmonary stenosis, patent ductus arteriosus, aortic stenosis and coarctation of the aorta. Congestive heart failure is the primary concern in infants with acyanotic lesions. The most common cyanotic lesions are tetralogy of Fallot and transposition of the great arteries. In infants with cyanotic lesions, hypoxia is more of a problem than congestive heart failure. Suspicion of a congenital heart defect should be raised by the presence of feeding difficulties in association with tachypnea, sweating and subcostal recession, or severe growth impairment. Follow-up of infants with congenital heart disease should follow the schedule of routine care for healthy babies with some modifications, such as administration of influenza and pneumococcal vaccines. More frequent follow-up is required if congestive heart failure is present. Family psychosocial issues should also be addressed. One of the main roles for the family physician is to help the parents put the diagnosis in perspective by clarifying expectations and misconceptions, and answering specific questions.

Diagnosis, Differential↗
PubMed1982

Respiratory syncytial viral infection in infants with congenital heart disease.

Occasional reports have suggested that infants with congenital heart disease may have an increased risk of severe illness from respiratory syncytial virus (RSV) infection. We prospectively studied 699 infants hospitalized during the winters of 1976 through 1980, when RSV was prevalent in the community; 229 of these infants had proved RSV infections acquired either before admission or during hospitalization; 27 had both congenital heart disease and RSV infection, and 46 had congenital heart disease without RSV infection. Infected infants with congenital heart disease had significantly more severe illness than those without congenital heart disease, as judged by the requirement for intensive care and assisted ventilation and by the mortality rate (37 per cent vs. 1.5 per cent, P less than 0.01). The infection was acquired nosocomially by 21 per cent of infected infants; the mortality rate from nosocomial infection was also higher in infants with congenital heart disease (44 per cent vs. 5 per cent, P less than 0.01). Pulmonary hypertension was the one condition particularly associated with severe RSV illness. Eight of the 11 infants (73 per cent) with congenital heart disease and pulmonary hypertension died during their RSV illness. The courses in infants with congenital heart disease with and without RSV infection were also compared. Their ages, types of cardiac lesions, and incidence of pulmonary hypertension were similar, but the infants with RSV infection had a higher mortality rate (37 per cent vs. 6.5 per cent, P less than 0.1).

Cross Infection↗
PubMed1987

Surgical management of difficult pacing problems in patients with congenital heart disease.

The child with congenital heart disease requiring permanent pacing presents a unique challenge with regard to the decision to pace, hardware, route, and unusual problems. Considerations of patient size, anatomy, insertion during and after complex intracardiac procedures, location of hardware, and unusual approaches are discussed including: placement of endocardial leads at open operation, closed transatrial endocardial technique, periprosthetic valvular endocardial placement, trans left superior vena cava placement and retromammary position via the axillary approach. Consideration of these unusual techniques may avoid frustration due to the complexities of the placement of pacing systems in these young patients.

Anticoagulants↗
PubMed2005

Improving the care of Canadian adults with congenital heart disease.

Canadian children with congenital heart disease (CHD) have been well cared for in the past half-century. These childhood success stories have resulted in there now being approximately 100,000 Canadian adults with CHD. Few of these patients have been cured or have normal hearts, and most were left with problems that will need to be addressed later in life. Approximately 55,000 such patients need expert care because their conditions are so rare or complicated that cardiologists and other caregivers have not been trained to look after them. Moreover, these 55,000 Canadians face premature mortality, the need for further surgery or the prospect of major complications. They need expert care to optimize the quality of their lives and to help them to avoid premature death. There is no plan for the care of these patients. When they reached 18 years of age, they were moved into an adult care system that does not understand them, does not care about them and has not provided for their care. Provincial and federal Ministries of Health have not yet taken an interest in these patients. Canadian professionals have taken important steps in support of adults with CHD. They established the Canadian Adult Congenital Heart Network, and they developed a National Care Plan and management guidelines for their care. They have also made important contributions to the scientific literature, advancing the care of these patients. Waiting lists are much too long for adult patients with CHD. There are serious local and regional obstacles to the care of these patients because there are no provincial or regional plans for their care. Cardiologists who treat adult patients with CHD cannot earn enough in a fee-for-service system. There are serious human resource problems in Canadian Adult Congenital Heart Network centres that need to be addressed. There also needs to be greater coordination and integration between the pediatric and adult systems that care for these patients. Adults with CHD desperately need the help of the Heart and Stroke Foundations, leaders in the Canadian health care community and, ultimately, the Ministries of Health to help them protect their health and access expert care when needed in a timely way. The promises offered to them as children must also be honoured in their adult years.

Adult↗
PubMed2002

Catheter interventions in adult patients with congenital heart disease.

Adult patients with congenital heart disease provide a wide variety of challenges for the interventional cardiologist. Procedures can broadly be divided into dilatation or closure. The most common interventions in our own practice are closure of atrial septal defects and patent foramen ovale, although closure of postoperative interatrial communications in Mustard, Senning, or Fontan patients is also possible. Transcatheter patent ductus arteriosus closure is also now routine, and occlusion of coronary artery fistulae can be safely attempted using coil embolisation. Balloon dilatation of pulmonary valve stenosis has excellent success rates, and dilatation or stenting of pulmonary artery stenoses is also beneficial in selected cases. Although aortic valve dilatation in adults has mixed results, dilatation or stenting of aortic coarctation is now becoming more widespread, and has promising results. Dilatation procedures may also be applicable to the postoperative patient with conduit or baffle stenosis.

Adult↗
PubMed1998

Electroanatomical mapping and ablation of the substrate supporting intraatrial reentrant tachycardia after palliation for complex congenital heart disease.

In patients with congenital heart disease who have undergone palliative surgical interventions postoperative arrhythmias frequently complicate the clinical course. Intraatrial reentrant tachycardias (IARTs) are one of the most common forms of postoperative arrhythmias in these patients and can lead to significant morbidity and even mortality. Drug therapy and/or antitachycardia pacing have been disappointing. Ablative therapy with radiofrequency energy offers a potential for cure for these patients but the conventional approach using multielectrode recordings and fluoroscopic guidance is technically difficult and provides limited success. Recent development of a novel nonfluoroscopic technology with electroanatomical mapping using the CARTO mapping/ablation system has shown promising results in defining the arrhythmia circuit, facilitating diagnosis, and guiding ablative therapy. Based on our preliminary experience, a systematic approach to postoperative IART using electroanatomical mapping is described. Further studies are needed to fully evaluate the impact of this new technology on the management and therapy of IART.

Adult↗
PubMed2005

Safety and efficacy of palivizumab prophylaxis in children with congenital heart disease.

BACKGROUND: Infants with congenital heart diseases (CHD) are at high risk for Respiratory syncytial virus (RSV) infection, which causes severe respiratory distress. Palivizumab, an anti-RSV monoclonal antibody, was licensed in the USA, Europe and Canada, and a large-scale placebo-controlled double-blind test in these countries confirmed its efficacy and safety. A survey using questionnaires to assess usage, prophylactic efficacy, and safety of palivizumab in Japanese infants and young children with CHD was conducted. METHODS: The survey was conducted between October 2002 and March 2003. The questionnaire asked for patients' characteristics, presence of CHD, underlying diseases, starting date and number of injection, adverse events, correlation between adverse events and treatment with palivizumab, and evaluation of efficacy. RESULTS: In total, 108 infants were reported from 61 institutions. A total of 60 of the 108 infants evaluated without major non-cardiac complications received intramuscular injection of 15 mg/kg per month of palivizumab in a manner not consistent with approved indications for this drug. A total of 43 cases (39.8%) had complexed CHD, while 64 cases (59.3%) had the first injection in October or November. The average number of injections was 3.0 +/- 1.4. Seven children (6.5%) had notable respiratory infections confirmed by positive test for RSV antigen, and five (4.6%) were hospitalized. No children died nor received mechanical ventilation. The number of adverse events was nine in five cases. There was no significant relationship between adverse events and treatment with palivizumab. CONCLUSION: Palivizumab is well-tolerated, fairly effective and safe in preventing severe RSV infection in infants and young children with CHD.

Antibodies, Monoclonal↗
PubMed1998

Increased energy expenditure in infants with cyanotic congenital heart disease.

Infants with cyanotic congenital heart disease (CCHD) often have reduced weight gain compared with infants in control groups. Our purpose was to conduct a longitudinal study of energy intake, resting energy expenditure (REE), and total energy expenditure (TEE) of a group of infants with CCHD. We hypothesized that increased REE and TEE and decreased energy intake in these infants would lead to reduced growth. Ten infants with uncorrected CCHD and 12 infants in a control group were studied at 2 weeks of age and again at 3 months. Indirect calorimetry was used to determine REE; the doubly labeled water method was used to determine TEE and intake. At 2 weeks and 3 months of age, infants with CCHD weighed significantly less than infants in the control group. No significant difference was seen in energy intake or REE between groups during either period. TEE was slightly but not statistically increased in the CCHD group at 2 weeks (72.6 +/- 17.4 vs 59.8 +/- 10.9 kcal/kg/d) and significantly increased at 3 months (93.6 +/- 23.3 vs 72.2 +/- 13.2 kcal/kg/d, P </=.03). We conclude that increased TEE but not increased REE is a primary factor in the reduced growth in infants with CCHD.

Calorimetry, Indirect↗
PubMed1999

Compression of the central airways by a dilated aorta in infants and children with congenital heart disease.

BACKGROUND: Children with congenital heart disease often experience respiratory symptoms in the preoperative and perioperative periods, which can complicate their management. An uncommon but important cause of respiratory insufficiency in such children is external airway compression. METHODS: We operated on 5 patients (median age, 6 months) with significant respiratory distress attributable to compression of the central airways by a dilated ascending aorta before or after repair of concomitant cardiovascular defects. Four of these patients had right aortic arch and 3 had pulmonary atresia with a ventricular septal defect and major aortopulmonary collaterals. In all patients, aortopexy was performed at the time of operation for the cardiovascular defects (n = 3) or after symptoms developed in the postoperative period (n = 2). The 3 patients in whom airway compression produced symptoms preoperatively also underwent reduction ascending aortoplasty. RESULTS: Symptoms resolved immediately after operation in 3 patients, whereas symptoms persisted in the other 2 patients and tracheostomy was required. At follow-up of 20 months to 5 years, all patients are alive and well, with mild or moderate respiratory symptoms in the 2 patients who required tracheostomy, both of whom were decannulated within 13 months. CONCLUSIONS: External airway compression can cause significant morbidity in patients with congenital heart defects other than vascular rings. In patients with respiratory symptoms in the context of a lesion that involves increased aortic outflow during intrauterine life and consequently, an enlarged ascending aorta, such as tetralogy of Fallot with pulmonary atresia, airway compression should be considered as a cause, especially if a right aortic arch is present or the patient also has pulmonary atresia with a ventricular septal defect and collaterals. Attempts to address this problem surgically may provide substantial relief, but increasing duration of airway compression is likely to lead to tracheal or bronchial malacia and persistent symptoms even after the compression is relieved.

Airway Obstruction↗