Alarming rate of liver cirrhosis after the small conduit extracardiac Fontan: A comparative analysis with the lateral tunnel.

BACKGROUND: The association between the prevalence of cirrhosis and the types of Fontan operations remains unclear. METHODS: We conducted a retrospective chart review of 332 patients who underwent the Fontan procedure at our institution. Four patients who underwent the atriopulmonary connection Fontan were excluded from the analysis. Patients who had intracardiac-extracardiac conduit (126) between 1989 and 2021 were pooled with those having extracardiac conduit (ECC) (134). The 260 patients who underwent the ECC and the 68 patients who had the lateral tunnel (LT) Fontan constitute the core of the study. RESULTS: Median age at the Fontan procedure was 23.7 months (interquartile range [IQR], 20.8-32.6) in the LT group, compared with 28.8 months (IQR, 24.6-39.5) in the ECC group (P < .01). The median follow-up was 14.8 years (IQR, 12.5-16.5) in the LT group and 7 years (IQR, 2.8-10.4) in the extracardiac conduit group. During the follow-up period, 3 patients (4.4%) with LT and 17 patients (6.5%) with ECC (11 patients with 16 mm or less conduit size) were diagnosed with cirrhosis. The prevalence of cirrhosis at 1, 5, 10, and 15 years was 0%, 0%, 0%, and 4.4% in the LT group, respectively, and 0%, 0.9%, 7.7%, and 29.8% in the ECC group (P < .01) Rates of mortality, Fontan revision, Fontan takedown, transplant, and complications were comparable between the 2 groups. CONCLUSIONS: The extracardiac conduit Fontan seems to be associated with faster development of cirrhosis.

Aortic valve-sparing procedure in the pediatric population.

Background: Aortic valve-sparing procedures have been established as a mainstream treatment option for adult patients with aortic root aneurysms. However, data regarding their use in the pediatric population is limited. This study reports on our experience with aortic valve-sparing procedures in children. Methods: A retrospective review was conducted of all patients who underwent an aortic valve-sparing procedure at the Royal Children's Hospital, Melbourne, Australia between April 2006 and April 2016. Clinical and echocardiographic data were analyzed. Results: The study included 17 patients, with a median age of 15.7 years and a majority of patients being male (82.4%). The most common diagnosis was transposition of great arteries after arterial switch operation, followed by Loeys-Dietz syndrome and Marfan syndrome. Preoperative echocardiography showed more than moderate aortic regurgitation in 94.1% of the patients. The David procedure was performed in all 17 patients, with no mortality during follow-up. Reoperation was required in 29.4% of patients, and 23.5% required aortic valve replacement. Freedom from reoperation for aortic valve replacement at 1, 5, and 10 years was 93.8%, 93.8% and 68.2% respectively. Conclusions: Aortic valve-sparing surgery can be successfully performed in the pediatric population. However, it requires a highly skilled surgeon due to the often dysplastic or distorted nature of these valves and the need for additional procedures on the aortic valve leaflets.

When to intervene the pulmonary artery: Importance of anatomical assessment in the diagnosis of pulmonary artery coarctation.

OBJECTIVE: Pulmonary artery coarctation (PACoA) is a major problem that increases the frequency of intervention. However, there is little evidence regarding the prediction of PACoA development. METHODS: A retrospective chart review was performed on 42 patients who underwent modified Blalock-Taussig shunt and preoperative contrast-enhanced computed tomography. An uneven PA branching was defined as an abnormal ductus arteriosus connection to the left PA distal to the PA branching on contrast-enhanced computed tomography. RESULTS: Nineteen (45.2%) of 42 patients were diagnosed with PACoA. The median diameters of the ductus on the aorta and PA sides were 4.1 mm and 3.6 mm in the PACoA group and 3.6 mm and 2.9 mm in the non-PACoA group, respectively (P = .07 and .28, respectively). Tortuous ductus was recognized in 7 (36.8%) patients in the PACoA group and 14 (60.8%) patients in the non-PACoA group (P = .12). PACoA was associated with pulmonary atresia (16 patients [84.2%] in the PACoA group and 12 patients [52.1%] in the non-PACoA group) (P = .02). All 19 patients had uneven PA branching in the PACoA group, whereas 5 of 23 (21.7%) patients had uneven PA branching in the non-PACoA group (P < .001). CONCLUSIONS: Uneven PA branching rather than the ductus arteriosus size was strongly associated with PACoA development; therefore, morphologic assessment by contrast-enhanced computed tomography should be considered in patients with pulmonary atresia.

Sinus plication technique for neoaorta dilation and regurgitation in patients with repaired congenital heart diseases.

We report 3 cases in sinus plication technique utilized to repair aortic or neoaortic root dilation and valve regurgitation after congenital heart disease operation.

Strategy of delayed repair of total anomalous pulmonary venous connection in right atrial isomerism and functional single ventricle.

Objective: Repair of total anomalous pulmonary venous connection (TAPVC) in neonates with right atrial isomerism and functional single ventricle is challenging. In our novel strategy, primary draining vein stenting (DVS) was applied to patients with preoperative pulmonary vein obstruction to delay TAPVC repair. This study investigated our initial experience with a strategy of delayed TAPVC repair, incorporating DVS. Methods: Twenty-nine patients with right atrial isomerism and functional single ventricle who had a severe obstruction in the course of draining veins, who required surgical or catheter intervention in their neonatal period were retrospectively reviewed (primary DVS: n = 11; primary TAPVC repair: n = 18). Results: Patients in the primary DVS group had more mixed type TAPVC (primary DVS: n = 5, 45.5%; primary TAPVC repair: n = 2, 11.1%; P = .03) and required more systemic to pulmonary shunt surgeries during their lifetime (primary DVS: n = 9, 81.8%; primary TAPVC repair: n = 6, 33.3%; P = .047). Kaplan-Meier analysis showed that primary DVS repair was associated with improved survival compared with primary TAPVC repair (survival rates at 90 days, 1 year, 3 years and 5 years: primary DVS: 100%, 80%, 68.6%, and 54.9%; primary TAPVC repair: 55.6%, 38.9%, 38.9%, and 38.9%, respectively [P = .04]). Of the 4 patients who underwent stenting of the ductus venosus, 3 had elevated liver enzymes after surgical repair of TAPVC due to ductus venosus steal, which markedly improved after coil embolization of the stent. Conclusions: For neonates with obstructive TAPVC and functional single ventricle, our delayed TAPVC repair using primary DVS appeared to improve survival compared with the conventional strategy.

Impact of pulmonary artery coarctation on pulmonary artery growth and definitive repair following modified Blalock-Taussig shunt.

OBJECTIVE: Pulmonary artery coarctation may pose a risk for pulmonary stenosis and subsequent failure to achieve definitive repair. We sought to assess the impact of pulmonary artery coarctation on pulmonary artery growth. METHODS: A retrospective chart review was performed in 130 patients, including 37 single ventricles with a modified Blalock-Taussig shunt as first palliation. Pulmonary artery coarctation was defined as discrete stenosis of the pulmonary artery, with a diameter of less than 3 mm and with the ductus arteriosus connected. Preoperative echocardiography showed pulmonary artery coarctation in 29 patients (22%). Concomitant pulmonary artery plasty was performed in 14 patients with discrete stenosis having a diameter of less than 2 mm. RESULTS: Pre-modified Blalock-Taussig shunt left pulmonary artery z-scores were lower in patients with pulmonary artery coarctation than in those without (-4.0 [-5.8, -2.1] vs -1.7 [-2.6, -0.8], P < .001), and this remained the same even after modified Blalock-Taussig shunt (-2.5 [-5.1, -0.5] vs -0.5 [-2.4, 0.8], P = .010). Concomitant pulmonary artery plasty did not result in catch-up growth of the left pulmonary artery (post-modified Blalock-Taussig shunt left pulmonary artery z-score in patients with pulmonary artery plasty: -3.0 (-6.5, -2.0) versus those without: -1.8 (-3.3, -0.3), P = .279). Definitive repair/Fontan completion was achieved in 111 patients (85%), and this was not affected by the presence of pulmonary artery coarctation. CONCLUSIONS: Pulmonary artery coarctation affected disproportionate pulmonary artery growth throughout the staged repair, but did not result in failure of definitive repair/Fontan completion. Pulmonary artery plasty during the neonatal period did not contribute to catch-up growth of the left pulmonary artery; therefore, surgical indications and timing should be carefully considered.

Concomitant aortic valve replacement, mitral valve replacement, and coronary artery bypass grafting for aortic stenosis and mitral regurgitation in a patient with relapsing polychondritis.

Relapsing polychondritis is a rare multi-system disease characterized by inflammation in cartilaginous structures and other connective tissues. Cardiovascular complications occur in 10-51% of the patients. We report a case of concomitant aortic valve replacement, mitral valve replacement, and coronary artery bypass grafting in a patient with relapsing polychondritis. A 71-year-old female with relapsing polychondritis on prednisolone (5 mg/day) for 15 years presented at our hospital for further evaluation of valvular disease. Severe aortic stenosis and severe mitral regurgitation were diagnosed. We performed aortic and mitral valve replacement. During surgery, we found connective tissue surrounding the intima of the sinus of Valsalva and stenosis of the right coronary artery ostium, which was not noted on preoperative coronary angiography. We removed the tissue and performed bypass grafting to the right coronary artery. Postoperative recovery was uneventful, and she was discharged 27 days after surgery.

Correction to: Concomitant aortic valve replacement, mitral valve replacement, and coronary artery bypass grafting for aortic stenosis and mitral regurgitation in a patient with relapsing polychondritis.

In the original publication of the article, the figures were published in wrong order.

Mitral valve repair versus replacement with preservation of the entire subvalvular apparatus.

OBJECTIVE: This study aimed to evaluate the outcomes of mitral valve (MV) repair versus MV replacement with preservation of the entire subvalvular apparatus. METHODS: We retrospectively searched our dedicated in-hospital database for patients who underwent MV surgery between 2012 and 2017. RESULTS: A total of 82 patients were divided into a group that underwent MV replacement (n = 35) and a group that underwent MV repair (n = 47). Patients undergoing MV replacement were significantly older (p < 0.01). Mortality at 30 days was not significantly different [MV replacement: n = 1 (2.9%), MV repair: n = 0 (0%); p = 0.43]. The single case of 30-day mortality after MV replacement was due to acute aortic dissection. The total cohort did not show significant differences in long-term survival (p = 0.07). There were no cardiac-related deaths in this cohort. Postoperative left ventricular end-diastolic diameter (MV replacement: 45.4 ± 6.2 mm, MV repair: 45.6 ± 5.8 mm; p = 0.89), left ventricular end-systolic diameter (MV replacement: 29.6 ± 7.1 mm, MV repair: 29.4 ± 5.2 mm; p = 0.89), and ejection fraction (MV replacement: 59.2 ± 11.4%, MV repair: 62.0 ± 6.8%; p = 0.17) were not significantly different. CONCLUSIONS: This study found that MV replacement had operative mortality, long-term survival, and complication rates similar to those of MV repair. There were no cardiac-related deaths in this cohort. MV replacement with preservation of the entire subvalvular apparatus does not seem to be inferior to MV repair.