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PubMed2004

[Family resiliency factors for the adaptation of family who have a congenital heart disease].

PURPOSE: The purpose of this study was to explore the relationships of family strain, perceived social support, family hardiness, and family adaptation and identify the family resiliency factors for the adaptation of families who have a child with congenital heart disease. METHOD: The sample consisted of 90 families who had a child diagnosed with congenital heart disease and completed surgical treatment. Data was collected from parents using a questionnaire. RESULTS: Results from path analyses revealed that family strain had a direct effect on both perceived social support and family hardiness, and an indirect effect on family adaptation. Also, the findings revealed that perceived social support had a direct effect on both family hardiness and family adaptation, and family hardiness had a direct effect on family adaptation. Thus, these results indicated that perceived social support and family hardiness had a mediating effect on family strain. CONCLUSION: Findings provide the evidence for the theoretical and empirical significance of perceived social support and family hardiness as family resiliency factors for family adaptation. Clinical implications of these findings might be discussed in terms of family-centered nursing interventions for the families who have a child with congenital heart disease based on an understanding of family resiliency for adaptation.

Adaptation, Psychological↗
PubMed2004

Outcomes of radiofrequency catheter ablation of atrioventricular reciprocating tachycardia in patients with congenital heart disease.

OBJECTIVES: Assessment of clinical outcomes of catheter ablation of atrioventricular reciprocating tachycardias in patients with congenital heart disease (CHD). BACKGROUND: Atrioventricular reciprocating tachycardias occur in patients with CHD and may be poorly tolerated. METHODS: Retrospective review of all 105 such ablations in 83 patients performed between 03/90 and 02/02 at one institution. RESULTS: The dominant arrhythmia mechanism was accessory pathway (70 patients, 84%), and the most common indications were drug-refractory tachycardia, life-threatening arrhythmia, and elective presurgical ablation. Congenital heart disease diagnoses were diverse, with one third of patients having Ebstein's anomaly. Twenty patients (24%) had catheter access limited by prior surgeries or occluded vascular access. Of 109 accessory pathways (APs), 74 (68%) were manifestly preexcited, and 71 (65%) were located on the right atrioventricular groove. Fourteen patients (20%) had multiple pathways. There were 2 major complications (1 death, 1 hemorrhage), and 3 minor complications (5.5% of procedures). Acute success rate was 80% per procedure, 82% for left- and 70% for right-sided APs. Acute success rates for patients with Ebstein's anomaly were similar to patients with other CHD diagnoses, but Ebstein's patients were more likely to have recurrence. At 44 +/- 35 months follow-up, successful ablation was achieved in 59% of procedures and 68% of patients, with 19 patients (23%) undergoing one or more repeat ablations. CONCLUSIONS: Compared to patients with normal cardiac anatomy, patients with CHD of all varieties have lower rates of acute and long-term success for ablation for atrioventricular reciprocating tachycardias.

Adolescent↗
PubMed2005

Validation and application of single breath-hold cine cardiac MR for ventricular function assessment in children with congenital heart disease at rest and during adenosine stress.

PURPOSE: To validate the accuracy of ventricular function analysis using a single breath-hold real-time steady sate free precession (SSFP) cine MR method and demonstrate its application during adenosine stress imaging in children with congenital heart disease. MATERIALS AND METHODS: Twenty-eight subjects with congenital heart disease were studied (mean age 12.4 +/- 2.3 years) with MR imaging at 1.5 T. Short-axis images covering the entirety of both ventricles were acquired at rest, with a conventional segmented SSFP cine sequence acquired over multiple breath-holds and a single breath-hold real-time SSFP sequence. Seventeen subjects were given an infusion of adenosine, and the single breath-hold real-time short-axis stack was repeated during stress. Two independent observers performed the ventricular function analysis. Data was compared between the 2 acquisition methods at rest and between the single breath-hold acquisition at rest and during adenosine stress. RESULTS: There was good agreement between the multiple breath-hold and single breath-hold methods for measurement of end-diastolic volume (r = 0.95 and 0.96, p < .0001) and end-systolic volume (ESV) (r = 0.76 and 0.90, p < .0001) for the left and right ventricles respectively, and the left ventricular mass (r = 0.97, p < .0001). Adenosine was administered safely to all subjects. During stress there were significant changes in the heart rate and ESV, which led to a significant increase in cardiac output (mean 1.5 +/- 1.0 L/min, p < .001). CONCLUSION: Single breath-hold real-time SSFP cine imaging is robust and accurate in assessing cardiac function in children with congenital heart disease. An application of this method is the rapid assessment of cardiac function during adenosine stress.

Adenosine↗
PubMed1996

Cine gradient-echo MR imaging and MR velocity mapping in the evaluation of congenital heart disease.

Cine gradient-echo (GRE) magnetic resonance (MR) imaging and MR velocity mapping can be applied to assess cardiovascular function and to measure volumetric flow in patients with repaired and untreated congenital heart disease. GRE images, particularly when displayed in a cine loop, are very useful for visualizing surgical anastomoses and conduits and for detecting stenosis, regurgitation, and left-to-right shunts. Multisection cine GRE imaging is a suitable method for measuring global right ventricular function, an important issue in congenital heart disease. MR velocity mapping is used for quantitative assessment of hemodynamics. Pressure gradients can be estimated from peak blood flow velocities in stenotic pulmonary arteries or surgical conduits. Because flow volume can be measured with MR velocity mapping, main-, left-, and right pulmonary artery flow, valvular regurgitation, left-to-right shunts, and ventricular filling can all be quantified. The abilities to elucidate complex cardiac anatomy and to measure cardiac function and flow in one examination make MR imaging a useful comprehensive tool for follow-up of congenital heart disease.

Adult↗
PubMed1989

Magnetic resonance imaging of congenital heart disease: sensitivity and specificity using receiver operating characteristic curve analysis.

MRI has shown promise for the evaluation of various congenital heart lesions. The current study was designed to assess the sensitivity and specificity of the technique for the evaluation of all major anatomic elements of the heart affected in simple and complex congenital heart disease. MR images of 51 patients (31 males and 20 females, ages 3 to 69 years) with a total of 110 congenital heart lesions were reviewed by a panel of two cardiac radiologists and one pediatric cardiologist, who assessed the cardiac pathoanatomy without knowledge of clinical details and assigned a confidence level to each diagnosis. The true diagnosis was established independently by the findings of angiocardiography and catheterization as well as by surgery, where applicable. ROC curves were generated from the responses, and the sensitivity at a specificity level of 90% was determined. MRI was shown to have a high sensitivity in evaluating great vessel relationships (100%), thoracic aorta abnormalities (94%), ASDs (91%) and VSDs (100%), visceroatrial situs (100%), and loop (100%). Three of five anomalies of the pulmonary and systemic veins were diagnosed by MRI. Right ventricular outflow obstructions (95%) were detected with a much higher sensitivity than lesions of the other valves (aortic valve 52%, mitral valve 62%, tricuspid valve 76%). Spin-echo MRI is a sensitive and specific method for the noninvasive assessment of congenital heart disease, with limitations in the evaluation of some valvular anomalies.

Adolescent↗
PubMed1983

Twenty-five years of progress in the medical treatment of pediatric and congenital heart disease.

In the past 25 years there has been a many-fold increase in the prospect that with early recognition and modern treatment the newborn with critical congenital heart disease will reach adult life in a healthy condition, prepared to earn a living and to function as a spouse and as a parent. Advancements in the medical treatment of congenital heart disease may create less public acclaim than may surgical treatment but many purely medical developments provide the basis for achieving ultimate surgical success and, by judicious use of some forms of medical treatment, operation can be avoided altogether. The eight major contributions to patient care that are discussed in this review and the 35 that are simply listed are merely examples of the many developments that have occurred in the past 25 years. These include: 1) the organization of pediatric cardiology and the contribution of volunteer health organizations, 2) continuing medical education aimed at promoting early diagnosis of congenital heart disease and prompt referral to a cardiac center, 3) advances in the technology of cardiac catheterization, 4) Rashkind's balloon atrial septostomy and other catheter manipulative procedures, 5) pharmacologic manipulation of the ductus, 6) beta-adrenergic blockade for control of a variety of problems, including paroxysmal hypoxemic attacks, certain arrhythmias and relief of symptoms in hypertrophic cardiomyopathy, 7) echocardiography, and 8) advances in arrhythmias, electrophysiologic studies and use of pacemakers.

Adrenergic beta-Antagonists↗
PubMed1995

[Adult patients with congenital heart diseases: Berne statistics].

Data of the 272 adult patients with congenital heart disease followed up in our department are analyzed. We emphasize the lack of reliable statistical surveys of this group of patients at the present time. The need for better intra- and interdisciplinary cooperation, specially between referring physicians and tertiary centers, is stressed.

Adult↗
PubMed1977

Congenital heart disease in de Lange's syndrome.

Seven (29%) of 24 patients with de Lange's syndrome were found to have congenital heart disease. Right-sided obstructive lesions were most commonly encountered. Approximately one in five of all patients with the de Lange syndrome exhibit congenital heart disease, usually ventricular septal defect or pulmonic stenosis.

De Lange Syndrome↗
PubMedDate not supplied

Noninvasive evaluation of newborns with congenital heart disease.

Over the last decade, diagnosis and management of neonates with congenital heart disease have been greatly influenced by the constant expansion of noninvasive methods. We classify the most common congenital defects presenting in the first 2 weeks of life, based on clinical presentation and hemodynamic characteristics, followed by a discussion of echocardiographic findings in the most common congenital heart lesions and how ultrasound techniques can help solve problems frequently encountered during the early postoperative period.

Echocardiography↗
PubMed2004

[Application of one-way valved patch in treatment of patients with congenital heart disease with severe pulmonary hypertension].

OBJECTIVE: To explore the effect of one-way valved patch used in congenital heart disease with severe pulmonary hypertension. METHODS: One-way valved patch was used in 30 patients of congenital heart disease with severe pulmonary hypertension (PP/PS > 0.75) in operation. Follow-up of 6 approximately 86 months was conducted to observe its effect. RESULTS: The pulmonary artery pressure was significantly decreased without trans-patch shunt in 11 cases postoperatively. Trans-patch shunt was determined in 27 cases within postperative 72 hours. There were 2 postoperative deaths out of these 27 patients: one died of low cardiac output syndrome 72 hours after operation, and the other died of right heart failure 4 weeks after operation. Thirty-six patients were restored to health and discharged. Three-month follow-up showed trans-patch shunt in 7 cases, including right-to left shunt in 4 cases and two-side shunt in other 3 cases. Color Doppler ultrasonography conducted 6 months after operation proved trans-patch shunt in 4 cases, right-to-left shunt in 1 case, two-side shunt in 2 cases, and left-to-right shunt in 1 case (PP/PS = 0.45). CONCLUSION: One-way valved patch is useful in selected patients in which postoperative right heart failure can be anticipated so as to shunt the blood in the right heart to the left heart and increase the blood volume in the left heart system to ensure the left heart output and minimize the risk of postoperative right heart failure, at the expense of systemic low oxygen saturation that is, however, well tolerated.

Adolescent↗
PubMed1982

Atrial right-to-left shunt in infants with respiratory and cardiac distress but without congenital heart disease. Demonstration by contrast echocardiography.

Contrast echocardiography was used to detect atrial right-to-left shunts in 20 term infants; there were 10 cyanosed infants who had severe respiratory distress and signs of cardiac failure but no apparent congenital heart disease and 10 noncyanosed infants with no respiratory distress and no signs of cardiac failure who underwent exchange transfusion for hyperbilirubinemia. All of the venous contrast echocardiograms in the cyanosed infants showed an atrial right-to-left shunt with a dense contrast effect in the left atrium and aorta but little in the right ventricle. A sparse effect in the left atrium and a dense one in the right ventricle were recorded in infants with resolving respiratory distress and in three infants from the control group. The other seven infants in the control group showed no right-to-left shunt. Conventional M-mode echocardiograms showed no evidence of congenital heart disease in any of the 20 infants. This contrast echocardiographic technique was useful for demonstrating an atrial right-to-left shunt in cyanosed term infants with neonatal respiratory problems and signs of congestive failure but no apparent congenital heart disease.

Cyanosis↗
PubMed1985

Early extubation after surgical repair of congenital heart disease.

Of 220 pediatric patients recovering from surgical repair of congenital heart disease, 147 (67%) met our criteria for early extubation and were extubated either in the operating room or within 6 h after admission to the surgical ICU. The duration of postoperative mechanical ventilation correlated with the duration of cardiopulmonary bypass during surgical repair. In patients undergoing repair of ventricular septal defect, preoperative measures of pulmonary vascular status (PPA/PAO and RP/RS) also correlated with the duration of mechanical ventilation.

Adolescent↗
PubMed1990

Late potentials and inducible ventricular tachycardia in surgically repaired congenital heart disease.

We compared signal-averaged electrocardiography with invasive electrophysiological study in patients after surgical repair of congenital heart disease to determine if potentially useful correlations exist between the two methods for assessment of risk for ventricular tachycardia. Thirty-one patients (age, 1-49 years; mean, 10.6 years) with congenital heart disease repaired with right ventriculotomy or postrepair right bundle branch block (77% postoperative tetralogy of Fallot) who had electrophysiological study were studied with signal-averaged electrocardiography. Patients were classified by electrophysiological study results as having no inducible ventricular tachycardia, nonsustained ventricular tachycardia, or sustained ventricular tachycardia. Signal-averaged electrocardiograms were examined for the duration of low-amplitude (less than or equal to 40 microV) QRS signal, duration of total QRS, and root-mean-square voltage of the terminal 40 msec of the QRS. Low-amplitude terminal root-mean-square voltage of 100 microV or less had 91% sensitivity and 70% specificity for ventricular tachycardia inducible by electrophysiological study. Similar sensitivity but less specificity were seen using the criterion of 20 msec or more total low-amplitude QRS signal (initial plus terminal) or using total QRS duration of 128 msec or more. There was a weaker association between terminal low-amplitude QRS signal of 15 msec or more and inducible ventricular tachycardia.(ABSTRACT TRUNCATED AT 250 WORDS)

Cardiac Pacing, Artificial↗
PubMed2002

Three-dimensional computed tomography in children with compression of the central airways complicating congenital heart disease.

PURPOSE: We investigated the quality and usefulness of spiral computed tomography and three-dimensional reconstruction in children with obstruction of the central airways as a complication of congenital heart disease. MATERIALS AND METHODS: Spiral computed tomography with three-dimensional reconstruction was performed in 49 children with obstruction of the central airways seen as a complication of congenital heart disease. Spiral scanning was performed during breathing in 40, and after sedation with chloral hydrate in 38. Contrast medium was administered through a pedal venous route in 42. We analyzed the motion artifact, additional information provided by, and clinical usefulness, of the three-dimensional images. We also investigated the factors influencing the quality of the images. RESULTS: Stenoses were seen in the trachea in 21 patients, and in bronchuses in 28. Their causes were an anomaly of the aortic arch in 6, posterior displacement of the aortic arch in 7, posterior displacement of the ascending aorta in 5, compression of the brachiocephalic artery in 5, absent pulmonary valve syndrome in 6, displaced or dilated cardiovascular structure in 17, and pulmonary arterial sling in 2. Motion artifact caused mild or negligible degradation of images in all patients except 6. Breath-holding in non-sedated children produced more severe motion artifact than did cardiovascular pulsation. Three-dimensional images provided additional information over two-dimensional images in 11, and provided clinically useful information in 10. Contrast injection via the pedal route was better for the quality of three-dimensional images than brachiocephalic injection (p = 0.013). CONCLUSIONS: Three-dimensional computed tomography is useful in evaluation of obstruction of the central airways in children with congenital heart disease. Despite the fact that motion artifact is unavoidable, the quality of three-dimensional images is acceptable for making a proper and accurate diagnosis. A pedal route is recommended for injection of contrast medium.

Adolescent↗
PubMed2001

Pharmacologic management of arrhythmias in patients with congenital heart disease.

The management of cardiac arrhythmias has evolved rapidly over the past decade. This includes the development of more effective antiarrhythmic medications as well as catheter- and device-based therapies. Antiarrhythmic medications remain the primary treatment modality for most acute arrhythmias; however, the long term use of these medications may be accompanied by severe adverse effects. For this reason, antiarrhythmic medications are increasingly used in conjunction with other forms of therapy, such as catheter ablation or pacemaker implantation. Patients with congenital heart disease often have an increased propensity for cardiac arrhythmias due to both inherent conduction system abnormalities and impaired ventricular function. The purpose of this review is to examine the currently available antiarrhythmic drugs and assess their role in the treatment of arrhythmias in patients with congenital heart disease. It is important to emphasize that patients with congenital heart disease often have hemodynamic limitations and may be at an increased risk for developing adverse effects with antiarrhythmic agents. An awareness of the arrhythmias associated with congenital heart disease, the natural history of these arrhythmias, and the potential benefit of treatment with antiarrhythmic medications versus other forms of therapy provides a rational basis for therapy in this challenging population of patients.

Anti-Arrhythmia Agents↗
PubMed1991

Congenital heart disease in Down's syndrome: two year prospective early screening study.

OBJECTIVE: To determine the effectiveness of clinical examination, chest radiography, and electrocardiography compared with echocardiography in detecting congenital heart disease early in the life of children with Down's syndrome. DESIGN: Prospective two year screening survey. SETTING: Regional paediatric cardiology service, Northern Ireland. PATIENTS: 81 newborn infants with Down's syndrome born in Northern Ireland between November 1987 and November 1989. INTERVENTIONS: Clinical examination, chest radiography, and electrocardiography soon after birth followed by cross sectional Doppler echocardiography. MAIN OUTCOME MEASURES: Diagnostic ability of clinical examination, radiography, and electrocardiography compared with echocardiographic findings. RESULTS: 34 babies had congenital heart disease detected by echocardiography (13 had atrioventricular septal defects, seven secundum atrial septal defects, six a solitary patent ductus arteriosus, five isolated ventricular septal defects, and three combinations of heart defects). Individual examination methods were insensitive (the sensitivity of clinical examination was 0.53, of radiography 0.44, and of electrocardiography 0.41) but highly specific (the specificity of clinical examination was 0.94, of radiography 0.98, and of electrocardiography 1.0), although sensitivity improved when the three techniques were combined (the sensitivity was 0.71, the specificity 0.91). CONCLUSION: Echocardiography performed early in life can detect congenital heart disease that might otherwise be missed. Early detection may help prevent complications such as pulmonary vascular disease that may adversely affect the outcome of cardiac surgery.

Down Syndrome↗
PubMed1998

Gastro-intestinal protein loss in late survivors of Fontan surgery and other congenital heart disease.

AIMS: Protein losing enteropathy is a serious complication of Fontan surgery. The aim of this study was to investigate gastro-intestinal protein loss in adults with congenital heart disease, both with and without Fontan surgery, and to correlate findings with systemic venous pressure. METHODS AND RESULTS: Forty eight patients were studied. The first group included adult survivors of Fontan surgery. The second and third groups were control patients with congenital heart disease who had not had Fontan surgery and had either normal or chronically elevated systemic venous pressure. Gastro-intestinal protein loss was assessed by measurement of faecal alpha-1-antitrypsin. Faecal alpha-1-antitrypsin levels were significantly higher in the Fontan group (0.55 +/- 0.15 mg. g-1 faeces, P = 0.002) and the control group with chronically elevated venous pressure (0.60 +/- 0.30 mg. g-1 faeces, P < 0.001) compared to the controls with normal venous pressure (0.29 = 0.12 mg. g-1 faeces). Of the 15 subjects who were found to have increased gastro-intestinal protein loss, only four had clinical protein-losing enteropathy. The degree of gastro-intestinal protein loss correlated significantly with venous pressure (P = 0.01) and with serum aspartate transaminase (P = 0.04). CONCLUSION: Increased gastro-intestinal protein loss is common in this select group of late survivors of Fontan surgery and in other subjects with congenital heart disease and chronic elevation of systemic venous pressure, and was present in patients who did not have protein-losing enteropathy. Increased faecal alpha-1-antitrypsin is an important finding in these patients as intervention at this stage, before the onset of florid protein-losing enteropathy, might prevent the development of further complications.

Adult↗
PubMed2006

Transition to adulthood in congenital heart disease: missed adolescent milestones.

PURPOSE: To determine whether adults with congenital heart disease (CHD) are better adjusted than adults with acquired heart disease (AHD). METHODS: Twenty-five adults with CHD completed the Patient Adjustment to Illness Self Report (PAIS-SR). RESULTS: CHD patients had good adjustment for Heath Care Orientation and Family Relations, but poorer adjustment for Vocational, Social and Domestic Environment, Sexual Relations, and Psychological Distress. CONCLUSIONS: The needs of CHD patients are not being met.

Adaptation, Psychological↗