We report the case of a 9-year-old male with severe congenital pulmonary valve stenosis referred to our centre for percutaneous valvotomy. On admission, trans-thoracic echocardiogram confirmed a unicuspid pulmonary valve with a peak/mean pulmonary valve gradient of 91/53 mmHg and a pulmonary annulus of 13.8 mm (-0.8 Z Score). It also showed an enlarged RV (RV/LV ratio 0,9). During cardiac catheterisation, an additional atrial septal defect (secundum) with significant left to right shunt (Qp/Qs > 2) was diagnosed, which was not amenable to percutaneous closure. The patient was referred for surgical repair.The atrial septal defect was closed by a direct running suture. The repair of the unicuspid valve consisted in bicuspidisation by a large commissurotomy to the left anterior wall of the pulmonary artery. The neo-commissure was created with two separate patches of autologous pericardium secured to the wall of the pulmonary root. The adjustment of the effective height of the pulmonary valve leaflets was done by trimming the patches and a triangular plication of the newly created posterior leaflet. Perioperative echocardiogram showed a peak gradient of 15 mm Hg and trivial pulmonary regurgitation. The total cross-clamp time was 92 min and the bypass time 123 min with a favourable evolution after the surgery.The particularity of the case is represented by the complexity of the bicuspidisation procedure. Using this technique, a tailored approach is needed for every patient.

UNASSIGNED: To compare the early operative outcome of TOF repair with three contemporary repair strategies of RVOTO repair i.e. TAP, Mono-cusp construction (MC) in TAP and pulmonary valve repair.
UNASSIGNED: Study is performed at Punjab Institute of Cardiology, Lahore from May 2016 to April 2020. Retrospective analysis of data was performed for patient who underwent TOF repair by three different strategies of RVOT repairs during TOF surgery based on z scoring for pulmonary valve annulus. Group-I underwent trans-annular patch repair, while Group-II and III underwent Mono-cusp repair with autologous pericardium and pulmonary valve repair respectively. Analysis of Variance (ANOVA) and Pearson Chi-Square (PCS) statistics were used to compare the three groups for numeric and categorical variables respectively. Post-hoc t-test and Bonferroni correction were performed for numeric data to compare two groups with each other. Chi-square test was used to perform comparison between groups for categorial variables.
UNASSIGNED: ANOVA for aortic cross clamp time, total CPB time, Post-operative mechanical ventilation time, ICU stay and hospital stay showed statistical difference among all three group with p-value less than 0.05 however post hoc T-test showed this variation is limited to post-operative mechanical ventilation only when groups compared with each other. PCS showed there was difference for incidence of difficult weaning from CPB when all three groups compared while there was no difference in operative mortality with p-value of 0.15. However, Group-II comparison with Group-I showed that weaning from CPB was superior in-Group-II with p-value of 0.016. Group-III showed the best statistics for all operative outcome variables among all three groups. Comparison of incidence of post-operative moderate pulmonary regurgitation before discharge between Group-II and Group-III showed significant difference with p-value of 0.0052.
UNASSIGNED: PV repair strategy should be employed for RVOT repair of TOF whenever feasible. MC repair showed fewer hours of postoperative mechanical ventilation and higher incidence of easy weaning from CPB when compared to TAP, however its impacts over ICU stay, Hospital stay and operative mortality is not profound in our TOF repair population.

In this review, we provide a brief description of recently published articles addressing topics relevant to pediatric cardiologists. Our hope is to provide a summary of the latest articles published recently in other journals in our field. The articles address (1) pulmonary valve repair in patients with tetralogy of Fallot and in patients with congenital pulmonary stenosis which seems to be a promising technique in selected patients, (2) the use of Losartan in patients with Marfan syndrome mostly in addition to beta blockers may result in a lower aortic root dilation rate and better clinical outcomes, (3) a summary of the characteristics of adults with congenital heart disease in the USA showed a wide variation in prevalence, associated morbidities, health care utilization and insurance type in different locations in the USA, (4) a large single center study confirmed that right ventricular morphology is associated with worse outcomes after the Fontan operation and that atrioventricular valve regurgitation is an important predictor of worse outcomes and precedes ventricular dysfunction post Fontan operation, (5) a large study describing the multi system inflammatory syndrome in children temporarily related to the COVID-19 pandemic in the USA showing that ventricular dysfunction is the most common cardiac manifestation (6) the reality of \"limping to transplantation\" showing that patients with one or more modifiable risk factors including mechanical ventilation, kidney or liver dysfunction are at significant increase risk post cardiac transplantation.

Pulmonary artery aneurysms are rare but are associated with a significant risk of rupture and dissection. Moreover, pulmonary valve regurgitation and/or stenosis often coexist. In this study, we present a case of a pulmonary artery aneurysm with severe pulmonary regurgitation in a patient with pulmonary hypertension treated with aneurysm resection and pulmonary valve repair.

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.