Early surgical closure of a large ventricular septal defect: Influence on long-term growth

AbstractThe pre- and postoperative growth patterns or 52 otherwise normal infants undergoing primary surgical closure of a large ventricular septal defect before 7 months of age were reviewed. Serial measurements of weight, length and head circumference were compiled for all patients preoperatively and in 46 long-term survivors and were expressed as Z scores (in standard deviations from the mean for age and gender).By the time of surgery at a mean age of 0.33 year, the mean weight, length and head circumference Z scores of all 52 infants were −2.9, −0.9 and −0.6, respectively, and were all significantly below normal (p < 0.001). At a mean age of 5.7 years, the mean weight, length and head circumference Z scores of 35 patients of normal birth weight were normal or varied only marginally from those of the reference population (−0.4, −0.1 and +0.5, respectively: p < 0.02, p > 0.05 and p = 0.008, respectively) and did not differ significantly in any variable from those of 44 normal siblings. However, among 11 infants with a low birth weight, all three variables remained abnormal at long-term follow-up when compared with the reference population (−1.7, −1.7 and −0.9, respectively; p < 0.001 for each) and 22 normal siblings (p < 0.008). The difference between pre- and postoperative Z scores was highly significant (p ≤ 0.004) for all three variables in the normal birth weight group, but only a significant difference in weight Z scores emerged in the low birth weight group (p < 0.001). Catch-up growth in most cases was complete within 6 to 12 months after operation.Early surgical repair of a large ventricular septal defect results in near normal long-term growth in the majority of patients. Residual growth disturbances are usually due to extracardiac factors. The present results support a policy of early surgical intervention in infants with a large ventricular septal defect

Patient-reported outcomes in the aging population of adults with congenital heart disease: results from APPROACH-IS.

The congenital heart disease (CHD) population now comprises an increasing number of older persons in their 6th decade of life and beyond. We cross-sectionally evaluated patient-reported outcomes (PROs) in persons with CHD aged 60 years or older, and contrasted these with PROs of younger patients aged 40-59 years and 18-39 years. Adjusted for demographic and medical characteristics, patients ≥60 years had a lower Physical Component Summary, higher Mental Component Summary, and lower anxiety (Hospital Anxiety and Depression Scale-Anxiety) scores than patients in the two younger categories. For satisfaction with life, older persons had a higher score than patients aged 40-59 years. Registration: ClinicalTrials.gov NCT02150603

Phenotypes of adults with congenital heart disease around the globe: a cluster analysis

Objective To derive cluster analysis-based groupings for adults with congenital heart disease (ACHD) when it comes to perceived health, psychological functioning, health behaviours and quality of life (QoL). Methods This study was part of a larger worldwide multicentre study called APPROACH-IS; a cross sectional study which recruited 4028 patients (2013–2015) from 15 participating countries. A hierarchical cluster analysis was performed using Ward's method in order to group patients with similar psychological characteristics, which were defined by taking into consideration the scores of the following tests: Sense Of Coherence, Health Behavior Scale (physical exercise score), Hospital Anxiety Depression Scale, Illness Perception Questionnaire, Satisfaction with Life Scale and the Visual Analogue Scale scores of the EQ-5D perceived health scale and a linear analogue scale (0–100) measuring QoL. Results 3768 patients with complete data were divided into 3 clusters. The first and second clusters represented 89.6% of patients in the analysis who reported a good health perception, QoL, psychological functioning and the greatest amount of exercise. Patients in the third cluster reported substantially lower scores in all PROs. This cluster was characterised by a significantly higher proportion of females, a higher average age the lowest education level, more complex forms of congenital heart disease and more medical comorbidities. Conclusions This study suggests that certain demographic and clinical characteristics may be linked to less favourable health perception, quality of life, psychological functioning, and health behaviours in ACHD. This information may be used to improve psychosocial screening and the timely provision of psychosocial care

Problem solving in paediatric medicine : an analysis of student and consultant diagnostic skills

Thesis (M.Ed.) - University of Melbourne, 1983A study was undertaken to examine the similarities among and differences between the process of problem solving by undergraduate medical students and experienced consultants in the area of paediatric medicine. To approximate the clinical situation, a naturalistic study was carried out. Patients were chosen from those attending a consultant medical outpatient department of a teaching hospital and selected because of their relatively common paediatric problems. A videotape recording was made of the patient- student/consultant encounter to facilitate subsequent analysis. The students/consultants were also asked to complete a prescribed protocol detailing their hypotheses or diagnoses at various stages of the interview and after completion of the examination. Their judgement as to how they had learned these skills in problem solving was also sought. Both students and consultants used a hypothetico-deductive method of problem solving, generating multiple hypotheses early within the patient encounter. However students, in contrast with the consultants, considered a greater number of hypotheses but did not, carry out an efficient inquiry strategy. They often arrived at an incorrect final diagnosis, despite having earlier considered the correct diagnosis. The experienced consultant on the other hand raised fewer. hypotheses, was more selective in his subsequent hypothesis testing and arrived at the final correct diagnosis. The consultants also seemed more understanding of the importance of the child within his family, which was particularly relevant in the assessment of the child with biopsycho-social symptoms. All students when reflecting on how they had learnt their problem solving skills, Included formal learning in their response. In contrast the experienced consultants all cited Independent learning. The observation of an appropriate clinical model was reported equally by both students and consultants. Discussion centres around these findings with additional consideration being given as to whether problem solving skills may be learnt

Major Complication Following Kawasaki Disease in an Infant—The Development of Apical Infarction and Aneurysm Formation

Considerable advances have occurred in the understanding of Kawasaki disease, with a substantial drop in morbidity and mortality following the infusion of gamma globulin during the acute phase. Nevertheless, major complications may still occur. A 27-year-old male presented as an infant of 11 weeks when he was diagnosed as having Kawasaki disease. He was appropriately treated with aspirin and a gamma globulin infusion following his diagnosis 5 days after the onset of his illness. Despite that, he went on to develop coronary aneurysms. He represented a few weeks later with a history of inconsolable crying associated with pallor, suggestive of ischaemic chest pain. A repeat echocardiogram revealed infarction of the apex of the left ventricle with localised thrombus formation. There were persistent aneurysms within both coronary artery systems. A further infusion of gamma globulin was given. In view of the thrombus formation, he was started on warfarin. The thrombus gradually resolved with the development of a clearly defined left ventricular apical aneurysm. He has remained on warfarin, aiming for an international normalised ratio (INR) level of 2 to 2.5. He developed mild left ventricular dysfunction during late childhood, which improved following the commencement of an angiotensin-converting enzyme (ACE) inhibitor. Despite his ventricular aneurysm, there has been no documented evidence of ventricular tachycardia over the years. Repeated testing initially by nuclear perfusion scans and then by stress echocardiograms failed to show any inducible ischaemia apart from the apical ventricular aneurysm. A recent computed tomography (CT) coronary angiogram revealed an ectatic origin of the left main and the right coronary arteries with mild calcification involving the mid-portion of the latter and slight calcification of the former. His raised cholesterol level has responded well to a statin. Despite the persistence of the ventricular aneurysm, he continues to be managed conservatively, as he has remained well. The question arises as to what the long-term implications are of his left ventricle apical aneurysm. Should it be excised? Is he at risk for ventricular tachycardia and sudden death? In addition, although the coronary aneurysms have resolved, the CT coronary angiogram shows calcium plaques in both coronary arteries at the site of the earlier aneurysms. This finding raises the question as to whether all children who develop coronary artery aneurysms following Kawasaki disease should have a CT coronary angiogram performed in adulthood

Ethical Issues in Fetal Management: A Cardiac Perspective

The ethical issues behind the management of a fetus with a serious abnormality and the decisions made in relation to the outcome of the pregnancy are complex. This reflective paper deals with the ethical principles of managing a pregnancy with a congenital anomaly, with particular emphasis on the fetus with a serious cardiac abnormality. One major ethical concern is whether the fetus is or is not independent being to whom obligations of beneficence are owed. We review the debate on this matter, and suggest that it is ethically more appropriate for physicians who are involved in management of fetal abnormality not to adopt and insist on their own position on this matter. Rather, the appropriate course is to respect the pregnant woman's own view of her fetus and how it should be regarded. This is an application of the principle of respect for autonomy. Within this framework, we discuss the difficulties in counselling a pregnant woman or expectant couple in this situation, and recommend three key steps in ethically sound counselling