Primary central pulmonary artery plasty for right atrial isomerism with pulmonary coarctation

Objective Patients with unbalanced pulmonary artery growth resulting from pulmonary coarctation are considered unsuitable candidates for the Fontan procedure. Particularly, patients with right isomerism pose a challenge. We aimed to investigate the use of primary central pulmonary artery plasty at initial palliation in patients with right isomerism. Methods We recruited 34 right isomerism patients with pulmonary atresia and pulmonary coarctation who underwent modified Blalock-Taussig shunt with or without primary central pulmonary artery plasty between 1998 and 2014. We classified them into the primary central pulmonary artery plasty (group P) and no primary central pulmonary artery plasty (group N) groups. We retrospectively analyzed reintervention for pulmonary artery after initial palliation, difference in size between the left and right pulmonary arteries, overall survival, success of the Fontan procedure. Results The group P and group N included 25 and 9 patients, respectively. Five (20%) and six (67%) patients in group P and group N, respectively, required reintervention for pulmonary artery after initial palliation ( p = 0.017). No patient underwent reintervention for the pulmonary artery before bidirectional cavopulmonary shunt in group P. There was a significant difference in the bilateral pulmonary artery size balance between the groups before bidirectional cavopulmonary shunt ( p = 0.041). The two-lung Fontan procedure was successful in 14 (56%) and 1 (11%) patient in group P and group N. Conclusion Primary central pulmonary artery plasty may contribute toward improving the balance in the size of the PA and preclude the need for reintervention for PA.

Pulsatile Bidirectional Cavopulmonary Shunt pada Anomali Ebstein Dewasa: Manajemen Perioperatif

Latar Belakang: Anomali Ebstein meliputi malformasi bentuk dan pergeseran katup trikuspid yang letaknya lebih ke arah apex dari ventrikel kanan. Tindakan tricuspid valve repair biasanya dipilih untuk tatalaksana definitif pada kasus ventrikel kanan yang adekuat, sedangkan bidirectional cavopulmonary shunt (BCPS) dipilih sebagai tindakan paliatif.Kasus: Pada pasien ini kelainan terdeteksi di usia dewasa, dimana memiliki prognosiskeluaran klinis yang lebih baik dibanding kasus neonatus. Pasien menunjukkan adanya penurunan aktivitas fisik, jantung berdebar, kelainan hepatomegali, dan kelainan bunyi jantung. Pemulihan pascaoperasi terbilang cukup sulit dan lama.Pembahasan: Ventrikel yang telah mengalami fibrosis dan dilatasi harus diantisipasi agar dapat mempertahankan hemodinamik yang baik. manajemen awal pascaoperasi difokuskan pada pengurangan afterload dan mempertahankan kontraktilitas ventrikel kanan guna mengoptimalkan stroke volume jantung kananKesimpulan: Manajemen pascaoperasi pada kasus ebstein sangat bervariasi dan amat bergantung pada derajat kelainan serta usia pasien. Teknik diagnostik, pembedahan, dan manajemen pascaoperasi yang lebih baik di era sekarang membuat keluaran klinis juga menjadi lebih baik.

Post-Fontan pulmonary artery growth in patients with a bidirectional cavopulmonary shunt with additional antegrade pulsatile blood flow

Background Patients with antegrade pulmonary blood flow after a bidirectional cavopulmonary shunt (Glenn) may have better pulmonary artery growth. This study evaluated pulmonary artery growth in patients with and without prior additional pulsatile antegrade flow in a Glenn shunt at midterm follow-up after a Fontan procedure. Methods We reviewed 212 patients who had single-ventricle palliation in a 10-year period;103 (33 in pulsatile group 1 and 70 in nonpulsatile group 2) were selected for analysis. Data on demographics, procedures, perioperative course, and midterm follow-up after the Fontan procedure were compared. Echocardiography data were collected. Pulmonary artery sizes measured at cardiac catheterization and follow-up echocardiograms were used to calculate the Nakata index. Results Perioperative details were comparable in both groups, mean pulmonary artery pressure and systemic oxygen saturations were higher in group 1 compared to group 2. Venovenous collaterals were increased in group 1. There was a significant difference in the pre-Fontan and follow-up Nakata index between groups. There was a significant increase in the Nakata index in group 1 between the pre-Glenn and pre-Fontan assessments as well as the Nakata index between the pre-Fontan and midterm follow-up. There was no significant change in the Nakata index in group 2 between assessments. Conclusions A pulsatile Glenn shunt is associated with better pulmonary artery growth which continues long after the additional pulsatile flow is eliminated. It is possible that the effects of anterograde pulmonary blood flow on pulmonary artery growth in early life continue long after the Fontan completion.