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PubMedDate not supplied

MR imaging and CT of vascular anomalies and connections in patients with congenital heart disease: significance in surgical planning.

To plan effective management of congenital heart disease, one needs the clearest understanding of the anatomy. Although echocardiography and angiography are the dominant imaging modalities in patients with congenital heart disease, magnetic resonance (MR) imaging and computed tomography (CT) are valuable noninvasive adjuncts. MR imaging and CT are effective in demonstrating the complex cardiovascular morphology present in congenital heart disease, especially the extracardiac morphology. In patients with tetralogy of Fallot with complex pulmonary artery anatomy, MR imaging and CT are useful in demonstrating the pulmonary artery anatomy, along with the significant aortopulmonary collateral vessels. In the heterotaxy syndromes, patients often have unusual atriovenous connections. MR imaging allows accurate identification of the hepatic, systemic, and pulmonary veins and their relationships to both atria. CT and MR are the imaging modalities of choice in a patient who is thought to have a vascular ring. Treatment of aortic coarctation is usually performed on the basis of typical clinical and echocardiographic findings. In patients with atypical clinical or echocardiographic findings, MR imaging and CT yield helpful information that can change the treatment plan. The enhanced preoperative understanding of congenital heart disease provided by MR imaging and CT simplifies surgical decision making and consequently may improve outcome.

Aortic Coarctation↗

PubMed1996

[Congenital heart disease surgery in Senegal. Indications, evaluation and perspectives].

In Senegal, congenital heart diseases (CHD) raise important issues. Their late detection makes more complicated the non yet resolved issue of the medico-surgical care. 108 patients with congenital heart diseases have been studied prospectively from February 1992 to May 1994. Epidemiological, clinical, paraclinical data are analysed. Prognosis and treatment are discussed. Congenital heart diseases represent 1% of the outpatient cardiovascular pathology. Diagnosis of the malformation based on echocardiography shows that most of congenital heart diseases are ventricular septal defect (25%); followed by Tetralogy of Fallot (13%). Average age of the patients is 6.86 years. 31% of the cases have heart failure. ECG is abnormal in 103 patients. On the radiologic side, average cardio-thoracic ration is 0.61. Because of heart failure, pulmonary hypertension and hypoxia, the spontaneous prognosis is cautious in 71 patients (66%) who should receive surgical treatment. Among them, 5 patients non operated died and 10 (14%) have been operated. Among the 10 patients who got surgery, 3 with wide persistent ductus arteriosus got in Dakar. All these patients have excellent operative results. In Senegal, only close heart surgery is available and prognosis of the vast majority of patients, including those with valvular and coronary heart diseases, is very cautious. Intervention of Europe-based aid organizations for evacuation is not useful. It is very important to promote, in Senegal, open heart surgery.

Adolescent↗

PubMed2002

Comprehensive care for adults with congenital heart disease: expanding roles for nurses.

Adults with congenital heart disease constitute a growing patient population. Although life expectancy of these patients is increasing, they may be confronted with specific medical, psychosocial and behavioural problems throughout their life span. Special attention needs to be given to patients' illness experiences; knowledge and health behaviour; employability and insurability. Hence, comprehensive care by specialised health care professionals, addressing the multidimensional problems experienced by the patients is required. Interdisciplinary teams in which nurses guarantee the management beyond typical medical issues are promising. Key elements of nurses' involvement are facilitation of the transition process from paediatric to adult cardiology, identification of patient needs, screening and referral for psychosocial problems, and education and counselling of patients and families. In particular, an advanced practice nurse seems to be pre-eminent to optimise illness management of adult patients with congenital heart disease.

Adaptation, Psychological↗

PubMed1988

Effect of pH and PCO2 on pulmonary and systemic hemodynamics after surgery in children with congenital heart disease and pulmonary hypertension.

Fourteen children with congenital heart disease and associated pulmonary hypertension (preoperative mean pulmonary artery pressure (MPAP) 48 mm Hg +/- 1 SEM were examined to determine the effect of arterial carbon dioxide tension (PaCO2) and pH on pulmonary and systemic hemodynamics after surgical repair. Baseline measurements were obtained with hyperventilation to PaCO2 20 to 30 mm Hg (pH 7.56 +/- 0.01 mm Hg). The addition of carbon dioxide to inspired gas to achieve a PaCO2 40 to 45 mm Hg (pH 7.35 +/- 0.01) resulted in a significant increase in MPAP, from 32 +/- 5 mm Hg to 47 +/- 8 mm Hg (p less than 0.05). An increase in mean cardiac index (CI) from 2.7 +/- 0.3 L/min/m2 to 3.3 +/- 0.3 L/min/m2 (p less than 0.05) explained in part the associated increase in MPAP. For a subgroup of eight patients with postoperative MPAP greater than 30 mm Hg (at pH 7.35 to 7.40), pulmonary vascular resistance index (PVRI) also significantly increased (p less than 0.05) as PaCO2 was increased, implying a direct pulmonary vasodilating effect of alkalosis. Removal of carbon dioxide from inspired gas returned hemodynamic values to baseline. The higher the MPAP at physiologic pH the greater the absolute amount of MPAP reduction and PVRI reduction (p less than 0.05) with alkalosis. No complications from alkalosis were seen. We suggest that a trial of hypocarbic alkalosis in the child with severe residual pulmonary hypertension after surgical repair of congenital heart disease is warranted to reduce right ventricular afterload.

Adolescent↗

PubMed2000

Mode of death in adults with congenital heart disease.

An increasing number of patients with congenital heart disease (CHD) are entering adulthood. Although prior studies have focused on the causes of death in the pediatric population, the modes of death for adults with CHD have not been well defined. In a cross-sectional study performed on a population of 2,609 consecutive adults assessed at a CHD specialty clinic, there were adequate information available in 197 of 199 deceased patients. Mean age at death was 37 +/- 15 years. Mortality was highest in patients with congenitally corrected transposition of the great arteries (26%), tricuspid atresia (25%), and univentricular connection (23%). Youngest mean age at death was observed in patients with tricuspid atresia (27 +/- 5 years), complete transposition of the great arteries (27 +/- 7 years), pulmonary atresia (27 +/- 6 years), and aortic coarctation (29 +/- 6 years). Sudden death (26%) was the most common cause of death followed by progressive heart failure (21%) and perioperative death (18%). Postmortem examinations were performed in 77 of 197 deceased patients (39%) and provided incremental data on the mechanism of death in 22% of autopsies. Thus, the 3 major causes of death in the growing population of adults with CHD are sudden, perioperative, and progressive heart failure.

Adolescent↗

PubMed1982

Phenytoin therapy for ventricular arrhythmias occurring late after surgery for congenital heart disease.

Sudden death late after surgery for congenital heart disease is usually attributed to ventricular dysrhythmias, which may be difficult to suppress. In this study 19 consecutive patients with ventricular premature complexes (VPCs) documented by 24-hour ambulatory ECG monitoring were treated with phenytoin orally. Sixteen patients had undergone previous repair of the tetralogy of Fallot; three had undergone aortic valve surgery. Nine of these children had been unresponsive to previous antiarrhythmic therapy. Before treatment, four patients had ventricular tachycardia, three had couplets, six had frequent multiform VPCs, four had infrequent multiform VPCs, and two had frequent uniform VPCs. During treatment with phenytoin, the arrhythmia was decreased in all 19 patients and was completely suppressed in 15; the four remaining patients had only uniform VPCs on repeat ambulatory ECG. The mean serum level was 16.8 micrograms/ml (range 12 to 25 micrograms/ml) with a mean dose of 3.4 mg/kg (range 2 to 4 mg/kg). In one patient a skin rash led to discontinuation of phenytoin; no other side effects occurred. In summary, phenytoin was used to successfully suppress ventricular dysrhythmias in 19 consecutive patients with VPCs late after surgery for congenital heart disease. Phenytoin would appear to be the drug of choice for this patient group.

Adolescent↗

PubMed1996

Expanded PTFE membrane to prevent cardiac injury during resternotomy for congenital heart disease.

BACKGROUND: Resternotomy for repair of congenital cardiac defects can result in cardiac injury. Closure of the pericardium during the initial operation may prevent this, and several pericardial substitutes have been tried, with variable results, in patients in whom primary pericardial closure is not possible. We conducted a multicenter observational study of the use of the expanded polytetrafluoroethylene membrane (Preclude Pericardial Membrane, formerly called the Gore-Tex Surgical Membrane; W. L. Gore & Associates, Flagstaff, AZ) in patients likely to undergo reoperation for treatment of congenital heart disease. METHODS: Data were collected retrospectively on all patients in whom the expanded polytetrafluoroethylene membrane was inserted at the initial operation for congenital heart disease at 12 centers in 1984 to 1993. RESULTS: A total of 1,085 patients (mean age, 55 +/- 2.5 months) received the membrane. During follow-up ranging from 1.3 to 10.5 years, 105 reoperations were performed. Injury during resternotomy occurred in only 1 patient (1% of reoperations). There were no membrane-related deaths or complications in the entire series of 1,085 patients. CONCLUSIONS: The expanded polytetrafluoroethylene membrane was safe and effective in helping to prevent cardiac injury during resternotomy for treatment of congenital heart disease.

Child, Preschool↗

PubMed2006

First trimester screening for congenital heart disease.

Most obstetrical practices in the United States offer pregnant women at least one ultrasound during pregnancy. This prenatal ultrasound is usually scheduled at around 18 to 20 weeks gestation. Both the American Institute of Ultrasound in Medicine and the American College of Obstetricians and Gynecologists recommend that the four-chamber view be included to screen for congenital heart disease. Recently, many investigators have attempted to screen for congenital heart disease earlier in pregnancy. Fetal nuchal translucency thickness traditionally used to screen for fetal aneuploidy by transvaginal and abdominal ultrasound has also been shown to identify a large proportion of fetuses with congenital heart disease. Recent studies have also reported congenital heart disease in chromosomally normal fetuses with increased nuchal translucency thickness in the first trimester. Advances in ultrasound technology and training over the last 10 years allow greater visualization rates of the four-chamber view and outflow tracks in the late first trimester (up to 90% visualization at 13 weeks). Fetal echocardiography in the first trimester may be indicated for fetuses at risk for congenital heart disease. In this article we present a review of the most recent and ongoing clinical and basic research to support an earlier first trimester approach to diagnosing congenital heart defects.

Adult↗

PubMed2001

Radiologic interpretation of congenital heart disease.

Radiology in the evaluation of congenital heart disease has changed over the years but still has an important role to play. Although we can rarely make the definitive diagnosis of the intracardiac abnormalities, we can direct the clinicians' attention when cardiac disease is unsuspected and we can assist in the evaluation of complications of the disease or its treatment.

Gestational Age↗

PubMed1950