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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↗
PubMedDate not supplied

Fetal diagnosis of fatal congenital heart disease.

The identification of the structure of the fetal heart became possible in the early 1980s as the resolution of ultrasound equipment improved. At that time, use of fetal echocardiography was confined to mothers whose fetuses were at increased risk of developing congenital heart disease, although only 10% of congenital heart disease occurs in this group. In 1985 a French group suggested that obstetricians involved in routine scanning be taught a simplified form of heart scanning, which made it possible to screen the general pregnant population for major anomalies. This form of heart scanning involved the examination of one section of the heart, the four-chamber view, and this approach greatly increased the potential impact of fetal echocardiography. As a result of the success of this program in both the United Kingdom and France, a continually increasing proportion of major cardiac defects are detected prenatally. There is almost no form of structural heart disease seen in a four-chamber projection during a routine scan that will not result in the eventual death of the child or young adult. Surgery may delay or defer this event, depending on the anatomic anomaly, but in general the outlook for affected children and their chance of reaching healthy adult life is poor. Most parents who learn this before 20 weeks of pregnancy will elect to interrupt the pregnancy. As a result a decline in the number of postnatal cases of some defects, such as the hypoplastic left-heart syndrome, can be documented in postnatal life.

Abortion, Eugenic↗
PubMed1987

Modification of pulmonary hypertension secondary to congenital heart disease by prostacyclin therapy.

We have shown that PGI2 is a powerful but not selective pulmonary vasodilator, and we believe that there is a role for PGI2 in pulmonary vascular disease secondary to congenital heart disease, but much work remains to be done, including comparisons of PGI2 with other vasodilators. The role of PGI2 in altering the cellular and chemical events producing pulmonary vascular disease secondary to congenital heart disease, and any role in long-term treatment, is largely unexplored.

Child↗
PubMed2001

Congenital heart disease in maternal phenylketonuria: report from the Maternal PKU Collaborative Study.

The frequency and types of congenital heart disease in offspring from pregnancies in women with hyperphenylalaninemia were examined in the international prospective Maternal Phenylketonuria Collaborative Study. Relationships of congenital heart disease in offspring to the basal blood phenylalanine level in the mother, metabolic control through diet during pregnancy, and phenylalanine hydroxylase mutations in mother and offspring were determined. The 416 offspring from 412 maternal phenylketonuria pregnancies that produced live births and 100 offspring from the 99 control pregnancies were included in this examination. Thirty-four of the 235 offspring (14%; 95% CI, 10.2 to 19.6%) from pregnancies in phenylketonuric women with a basal phenylalanine level > or = 900 microM (15 mg/dL) [normal blood phenylalanine < 120 microM (2 mg/dL)] and not in metabolic control [phenylalanine level < or = 600 microM (10 mg/dL)] by the eighth gestational week had congenital heart disease compared with one control offspring (1%) with congenital heart disease. One offspring among the 50 (2%) from mothers with non-phenylketonuria mild hyperphenylalaninemia also had congenital heart disease. Coarctation of the aorta and hypoplastic left heart syndrome were overrepresented compared with expected percentages among those with congenital heart disease in the general population. A basal maternal phenylalanine level > 1800 microM (30 mg/dL) significantly increased the risk for bearing a child with congenital heart disease (p = 0.003). Phenylalanine hydroxylase mutations in the mothers and offspring did not have an independent relationship to congenital heart disease but were related through the basal maternal phenylalanine levels. The data in this study indicate that a basal maternal phenylalanine level of 900 microM may be a threshold for congenital heart disease, that women with the most severe degree of phenylketonuria are at highest risk for bearing such a child, and that prevention of the congenital heart disease requires initiation of the low phenylalanine diet before conception or early in pregnancy with metabolic control no later than the eighth gestational week.

Female↗
PubMed2006

[Role of interventional catheterization in adults with congenital heart disease].

The advances in surgical and interventional treatment of congenital heart diseases have allowed a large number of patients with congenital heart disease to attain the adult age. This population involves almost 0.3/1000 of the total population in Western Europe and North America and can be estimated to be 25,000 patients in Italy. Patients with operated tetralogy of Fallot, benign forms of pulmonary atresia with ventricular septal defect, simple or complex transposition of the great arteries usually survive beyond childhood. These patients may need repeat interventions to treat lesions of native or reconstructed pulmonary arteries and/or aortic arch, to occlude residual shunts, to treat pulmonary incompetence. More complex heart diseases such as single ventricle, rarely allow survival until the adult age. The majority of these patients undergo heart transplant, often made difficult by multiple cardiac surgical interventions, anomalies of the pulmonary arteries, chronic cyanosis, and aorto-pulmonary shunts. Patients with relatively simple or complex congenital heart diseases need to be followed in specialized units, like those existing in the United States, Canada, and Great Britain.

Adult↗
PubMed2006

Current patterns of infective endocarditis in congenital heart disease.

OBJECTIVE: To assess the changing profile of infective endocarditis in patients with congenital heart disease. METHODS: All cases diagnosed from 1966 to 2001 (revised Duke criteria) were retrospectively reviewed and categorised in periods I (< 1990) and II (>or= 1990). RESULTS: 153 episodes occurred, 81 in period I and 72 in period II. Mean age of affected patients was higher in period II. Non-operated ventricular septal defect, Rastelli correction and palliated cyanotic heart disease increased. Infective endocarditis in corrective surgery changed to patients with prosthetic material. Post-surgical cases decreased. Dental problems were the leading cause (period I 20% v II 33% of cases) with a large variety of pathological organisms (multiple species of Streptococcus). Cutaneous causative infections increased (5% to 17%) with different species of Staphylococcus. Negative blood cultures lessened (20% to 7%, p = 0.03). Streptococci were the most common causative organisms in both periods. Severe heart failure and cardiac complications lessened (20% to 4% and 31% to 18% during periods I and II, respectively). Early surgery was more frequent in period II (32% v 18.5%, p = 0.02). One- and 10-year survival was 91% v 97% in period I and 89% v 97% in period II, respectively (NS). CONCLUSION: Current targets include complex cyanotic disease, congenital heart disease corrected with prosthetic material and small ventricular septal defect. Postoperative cases lessened; dental and cutaneous causes increased. Survival was unchanged. Prophylactic measures targeted at dental and cutaneous sources should be emphasised.

Adult↗
PubMed1995

Pregnancy in the woman with congenital heart disease.

Advances in the diagnosis and treatment of congenital heart disease have improved survival for children born with complex congenital lesions. As the female patient enters adulthood, questions regarding the safety and feasibility of pregnancy arise. The hemodynamic changes of pregnancy may be well-tolerated by some patients and yet quite challenging to others. Complications may be anticipated based upon residual anatomic defects, type of surgery and sequelae of surgery. Specific congenital lesions are discussed with respect to hemodynamic changes of pregnancy in both the uncorrected and corrected states. Due to advances in diagnostic modalities, congenital heart disease may first be diagnosed in the adult patient during pregnancy. The risk of pregnancy in these women has not yet been completely defined as there is not a large experience regarding the more complicated lesions. Women should have a thorough preconception evaluation and be counselled about their potential risks. They should also be counselled that their fetus has an increased risk of congenital heart disease. Management during pregnancy, labor and delivery depends upon the specific cardiac problem. It must be individualized for the patient and formulated from a multidisciplinary approach involving obstetrics, cardiology and anesthesia.

Adult↗
PubMed1982

Continuous enteral feedings. An important adjunct to the management of complex congenital heart disease.

Eleven infants with complex congenital heart lesions were given continuous enteral infusions after failure to gain weight adequately despite use of hypercaloric formulas and nutritional supplementation. Formulas used before institution of enteral feedings were continued. Dietary and caloric intake and weight measurements were obtained at weekly or monthly intervals. Both mean daily caloric intake and mean daily weight gain were greater after initiation of continuous enteral feedings. Rate of weight gain improved in all 11 children after institution of enteral feedings. Continuous enteral feeding increases weight gain in babies with complex congenital heart disease, allowing earlier and safer surgical intervention.

Body Weight↗
PubMed1989

[Pregnancy in women with congenital heart disease].

During the past decades, the incidence of pregnant women with heart disease in Denmark has decreased from 0.9% to 0.3%. Heart disease in connection with pregnancy still remains the commonest non-obstetric cause of death. Previously, rheumatic valvular heart disease constituted the majority of all forms of heart disease in pregnant women. At present, congenital heart disease constitutes at least 80% of the maternal heart disease. Progress in heart surgery has had the result that increasingly more complicated forms of cardiac deformities can be corrected so that more survive to adult life. The range of cardiac deformities has therefore increased in recent accounts concerning pregnant women with heart disease. Ventricular septum defect, atrial septum defect and persistent ductus arteriosus still constitute approximately 50% of the forms of heart disease in pregnant women. In Denmark, the incidence of congenital heart disease is approximately 0.6% but between 2.5% and 4.2% of infants of women with congenital heart disease also have heart disease. In counselling about the risks in pregnancy, the NYHA scale provides a good guideline. Alle conditions with pulmonary hypertension are contraindications for pregnancy. The greatest risk for death occurs early in the puerperium. Guidelines for control and treatment of pregnant women with heart disease are presented. It is concluded, that pregnant women with heart disease should be assessed and be followed up in centres with the necessary cardiac, obstetric and anaesthesiologic expertise.

Denmark↗
PubMed1978

The pulmonary circulation in congenital heart disease. II. Pulmonary hypertension.

In young children with congenital heart disease the pulmonary circulation is exposed to abnormal haemodynamic conditions before it is fully developed. In the newborn infant the persistence or development of pulmonary hypertension rapidly leads to structural change. The speed with which an increase in muscularity can develop has hitherto been underestimated. In most children dying in early infancy with congenital heart disease and pulmonary hypertension the presence of thick walled small arteries is due not to persistence of the high wall thickness of foetal life, but to a rapid postnatal response of the pulmonary circulation to pulmonary hypertension. In older patients with a ventricular septal defect, aged between 3 months and 4 years, the presence of pulmonary hypertension has been shown to interfere with the growth and development of the pulmonary circulation, judging this by reduction in size and multiplication of intra-acinar arteries and an increase in muscularity of both pre and intra-acinar arteries and veins. In these patients elevation of pulmonary vascular resistance was associated with failure of the intra-acinar pulmonary circulation to develop normally and not with obliterative pulmonary vascular disease. Recent studies indicate that growth and development of the peripheral pulmonary circulation can be quantitated in lung biopsies taken from infants and young children with congenital heart disease. It should therefore be possibe to correlate structure and function at a critical period of lung development, before the changes of obliterative pulmonary vascular disease are established.

Aortic Coarctation↗
PubMed2003

Unusual stents in infants with severe congenital heart disease.

Two infants with severe congenital heart disease requiring intervention are described. Although both required conventional surgical therapy, mitigating circumstances led to catheter intervention for short-term palliation. The feasibility and advisability of such procedures is discussed.

Cardiac Catheterization↗
PubMed1998

Cardiac transplantation for pediatric patients. With inoperable congenital heart disease.

Recent studies have reported the expanding use of transplantation as the definitive option for pediatric patients with inoperable congenital heart disease. This study compares perioperative risk factors and outcomes in pediatric patients who received heart transplants for congenital heart disease with those in pediatric patients who received heart transplants for cardiomyopathy. Retrospective data collected on 40 consecutive pediatric patients undergoing cardiac transplantation from 1 January 1990 through 31 January 1995 provided the following results: 26 patients with cardiomyopathy (mean age, 7.6 years) and 14 patients with congenital heart disease (mean age, 7.2 years) underwent heart transplantation. Between groups, no significant difference was detected in waiting time for a donor heart (cardiomyopathy = 85 days, range = 2 to 409; congenital heart disease = 126 days, range = 9 to 396; P = NS); in donor/recipient weight ratio (1.27 +/- 0.34 vs 1.27 +/- 0.28, P = NS); or in ischemic times (209 +/- 92 minutes vs 248 +/- 70 minutes, P = NS). Cardiopulmonary bypass times accounted for the only significant difference (73 +/- 21 minutes vs 102 +/- 29 minutes, P = 0.003). No significant difference was found in the number of infection episodes, total days hospitalized, rejection episodes, or incidence of transplant coronary artery disease. Forty-month actuarial survival was 88% +/- 6% and 92% +/- 7% for cardiomyopathy and congenital heart disease transplant recipients, respectively (P = NS). We conclude that post-transplantation morbidity and mortality in patients with previous congenital heart disease are not significantly different from morbidity and mortality in patients with cardiomyopathy. Transplantation should be considered an acceptable therapeutic option for patients with congenital heart disease when surgical repair of the native heart is not possible.

Adolescent↗
PubMed2004

Development gone awry: congenital heart disease.

Significant advances in the understanding of the molecular and genetic basis of congenital heart disease have emerged from gene inactivation studies in mice and from human genetic investigations. However, the ability to utilize information gleaned from animal models to inform clinical care of patients depends on an accurate anatomic analysis and presentation in terms that are meaningful to the clinical pediatric cardiologist. Likewise, the enormous depth and breadth of accumulated clinical experience can inform the developmental biologist and can highlight the importance and interrelationships of particular phenotypes. The explosion of potentially informative genetic tools demands that basic scientists and clinicians concerned with congenital cardiac disease enhance the ongoing bidirectional dialogue. In some cases, categories of congenital disease familiar to clinicians are not recognized by developmental biologists, and mechanisms accepted by the biologist seem inconsistent with clinical experience. In this review, we summarize some of the more clinically significant forms of congenital heart disease, and we highlight relevant genetic and developmental pathways.

Animals↗
PubMed2006

The anesthesiologist's role in adults with congenital heart disease.

Anesthesia for adults with congenital heart disease has many challenging physiologic considerations. Collaborative relationships of a multidisciplinary team including cardiology, cardiac surgery, anesthesiology, and intensive care are essential to ensure positive outcomes in this population for noncardiac and cardiac surgery.

Adolescent↗
PubMed1994

Respiratory failure in congenital heart disease.

Infants and children with congenital heart disease (CHD) present unique difficulties when they develop respiratory failure, either as a consequence of their heart disease, in relation to cardiac surgery, or from infectious causes. Extensive cardiac surgical repairs are now being performed on younger infants with complex anatomy and physiology. The evolution of cardiac surgical technique and perioperative management has revealed the importance of subtle interactions between respiratory physiology and hemodynamic performance.

Child↗
PubMed2002

Effects of autologous platelet concentrate reinfusion after open heart surgery in patients with congenital heart disease.

BACKGROUND: Plasma pheresis and reinfusion of platelet-rich plasma has not been shown to reduce blood loss in cardiac patients. Recently, freshly prepared autologous platelet concentrates (PC) can be made from patient's blood and has a higher concentration than conventional platelet rich plasma. In this study, the effects of autologous PC reinfusion were examined after open heart surgery in patients with congenital heart disease. METHODS: Eight patients with noncyanotic congenital heart disease, who underwent open heart surgery and reinfusion of autologous PC, were classified as the PC group. Eight other patients with noncyanotic congenital heart disease, who underwent only open heart surgery, were defined as the control group. Ages ranged from 2 to 24 years and were not significantly different between the two groups (9.3 +/- 5.1 years in the PC group and 12.6 +/- 7.9 years in the control group, p = 0.33). In the PC group, blood was collected from the femoral vein through a 6F catheter introducer; 9 to 20 U (13.0 +/- 5.4 U, 0.42 +/- 0.22 U/kg) of autologous PC were prepared and were reinfused after protamine administration. The time course of platelet counts was examined until postoperative day 7. Aggregation responses to adenosine diphosphate; (4 micromol/L and 8 micromol/L), collagen (1 micromol/L and 5 micromol/L), and epinephrine (5 micromol/L and 10 micromol/L) were evaluated after induction of anesthesia (individual references), after protamine administration, at the end of the operation; these responses were shown as recovery ratios. RESULTS: Blood loss during surgery in the PC group was significantly less than in the control group (4.8 +/- 3.0 mL/kg versus 7.8 +/- 1.7 mL/kg, p = 0.044). Similarly blood loss on postoperative day 1 in the PC group was significantly less than in the control group (3.6 +/- 1.2 mL/kg versus 7.2 +/- 3.1 mL/kg, p = 0.013). The platelet counts in the PC group were larger than those in the control group until postoperative day 5, after reinfusion of prepared autologous PC. The recovery ratios of the aggregation responses to adenosine diphosphate, collagen, and epinephrine after protamine administration were not significantly different between the two groups. However, recovery in the PC group after reinfusion of the prepared autologous PC was greater than in the control group. CONCLUSIONS: Reinfusion of the freshly prepared autologous PC was followed by good aggregation responses and low blood loss in patients with noncyanotic congenital heart disease after open heart surgery. This procedure may be useful in pediatric open heart surgery without blood transfusion or with little administration of homologous blood products.

Adenosine Diphosphate↗
PubMed1986

Importance of coronary artery anomalies in operations for congenital heart disease.

A congenital coronary artery anomaly influenced operative management of 21 children (34 operations) during a recent 8-year interval. This group represented 1.3% of cardiothoracic operations performed. Survivors included 5 of 10 children having correction of the left coronary artery from the pulmonary artery and 11 children having correction of intracardiac defects associated with major coronary arteries crossing the right ventricular outflow tract. Two children survived primary repair of injured major coronary arteries. Isolated coronary artery anatomy must be considered during management of intracardiac defects. Intraoperative injury to a major coronary artery requires meticulous repair.

Adolescent↗