Constrictive pericarditis: rare but reversible cause of protein losing enteropathy.

Constrictive pericarditis is uncommon in children. It results from scarring and consequent loss of the normal elasticity of the pericardial sac and is most commonly seen as a late sequelae of idiopathic or viral pericarditis. Here, we report a case of protein losing enteropathy as a complication of constrictive pericarditis in a 2-year-old child. Pericardial thickening was demonstrated by cardiac MRI and subsequent pericardiectomy led to remarkable improvement and resolution of protein losing enteropathy.

Three-Dimensional Virtual and Printed Prototypes in Complex Congenital and Pediatric Cardiac Surgery-A Multidisciplinary Team-Learning Experience.

Three-dimensional (3D) virtual modeling and printing advances individualized medicine and surgery. In congenital cardiac surgery, 3D virtual models and printed prototypes offer advantages of better understanding of complex anatomy, hands-on preoperative surgical planning and emulation, and improved communication within the multidisciplinary team and to patients. We report our single center team-learning experience about the realization and validation of possible clinical benefits of 3D-printed models in surgical planning of complex congenital cardiac surgery. CT-angiography raw data were segmented into 3D-virtual models of the heart-great vessels. Prototypes were 3D-printed as rigid "blood-volume" and flexible "hollow". The accuracy of the models was evaluated intraoperatively. Production steps were realized in the framework of a clinical/research partnership. We produced 3D prototypes of the heart-great vessels for 15 case scenarios (nine males, median age: 11 months) undergoing complex intracardiac repairs. Parity between 3D models and intraoperative structures was within 1 mm range. Models refined diagnostics in 13/15, provided new anatomic information in 9/15. As a team-learning experience, all complex staged redo-operations (13/15; Aristotle-score mean: 10.64 ± 1.95) were rehearsed on the 3D models preoperatively. 3D-printed prototypes significantly contributed to an improved/alternative operative plan on the surgical approach, modification of intracardiac repair in 13/15. No operative morbidity/mortality occurred. Our clinical/research partnership provided coverage for the extra time/labor and material/machinery not financed by insurance. 3D-printed models provided a team-learning experience and contributed to the safety of complex congenital cardiac surgeries. A clinical/research partnership may open avenues for bioprinting of patient-specific implants.

Pulmonary valve restitution following transannular patch repair of tetralogy of Fallot.

Chronic pulmonary insufficiency following transannular patch repair of tetralogy of Fallot may mandate restoration of a competent pulmonary valve. Pulmonary valve leaflets that are preserved at initial surgery may grow and develop normal morphology and subsequent valve repair may be possible. We reviewed our experience with native pulmonary valve restitution following transannular patch repair (2001-15). The cohort included 9 patients with a median age of 18.7 (range 10.6-31.3) years. Operative technique involved reapproximation of the anterior commissure of the pulmonary valve. Median length of stay was 3 days, and there were no deaths. At median follow-up of 2.0 (0.4-13.5) years, pulmonary insufficiency was graded as ≤mild (n = 4), mild-moderate or moderate (n = 4) and moderate-severe (n = 1); pulmonary stenosis was ≤mild (n = 8) and moderate (n = 1), with median peak gradient of 21 (16-64) mmHg. No patient required reintervention. At reoperation to treat pulmonary insufficiency in repaired TOF, if residual leaflets are found with favourable anatomy, restitution of the native valve should be considered. This valve-preserving technique avoids the certain failure of a bioprosthesis and is associated with favourable early outcomes. The viability of this option may influence surgeons to leave the pulmonary leaflets in situ at the time of initial repair.

Infective endocarditis prophylaxis: current practice trend among paediatric cardiologists: are we following the 2007 guidelines?

BACKGROUND: In 2007, the American Heart Association modified the infective endocarditis prophylaxis guidelines by limiting the use of antibiotics in patients with cardiac conditions associated with the highest risk of adverse outcomes after infective endocarditis. Our objective was to evaluate current practice for infective endocarditis prophylaxis among paediatric cardiologists. METHODS: A web-based survey focussing on current practice, describing the use of antibiotics for infective endocarditis prophylaxis in various congenital and acquired heart diseases, was distributed via e-mail to paediatric cardiologists. The survey was kept anonymous and was distributed twice. RESULTS: Data from 253 participants were analysed. Most paediatric cardiologists discontinued infective endocarditis prophylaxis in patients with simple lesions such as small ventricular septal defect, patent ductus arteriosus, and bicuspid aortic valve without stenosis or regurgitation; however, significant disagreement persists in prescribing infective endocarditis prophylaxis in certain conditions such as rheumatic heart disease, Fontan palliation without fenestration, and the Ross procedure. Use of antibiotic prophylaxis in certain selected conditions for which infective endocarditis prophylaxis has been indicated as per the current guidelines varies from 44 to 83%. Only 44% follow the current guidelines exclusively, and 34% regularly discuss the importance of oral hygiene with their patients at risk for infective endocarditis. CONCLUSION: Significant heterogeneity still persists in recommending infective endocarditis prophylaxis for several cardiac lesions among paediatric cardiologists. More than half of the participants (56%) do not follow the current guidelines exclusively in their practice. Counselling for optimal oral health in patients at risk for infective endocarditis needs to be optimised in the current practice.

Teenage heart murmurs.

The first-time appearance of a murmur in an adolescent can create a substantial amount of anxiety in the parents and the teenager. The appropriate evaluation and diagnosis is very important in decision-making regarding sports participation in this population. Accurate identification of the innocent murmurs can obviate the need for echocardiography. Identification of a pathologic murmur may reduce morbidity and, possibly, mortality in critical lesions such as hypertrophic cardiomyopathy. This article discusses the physiology and characteristics of different murmurs, and outlines an approach to cardiac murmurs in adolescents.