scholarly journals Apex rotation as a risk factor for total anomalous pulmonary connection repair in single ventricle

2021 ◽  
Author(s):  
Hidetsugu Asai ◽  
Yasushige Shingu ◽  
Jin Ikarashi ◽  
Yuchen Cao ◽  
Daisuke Takeyoshi ◽  
...  
Keyword(s):  
Risk Factor ◽  
Vertebral Body ◽  
Single Ventricle ◽  
Right Atrial ◽  
Pulmonary Venous Obstruction ◽  
Sutureless Technique ◽  
High Incidence ◽  
Atrial Isomerism ◽  
Anomalous Pulmonary Venous Connection

Background: The high incidence of postoperative pulmonary venous obstruction (PVO) is a major mortality-associated concern in patients with right atrial isomerism and extracardiac total anomalous pulmonary venous connection (TAPVC). We evaluated new anatomical risk factors for reducing the space behind the heart after TAPVC repair. Methods: 18 patients who underwent TAPVC repair between 2014 and 2020 were enrolled. Sutureless technique was used in 12 patients and conventional repair in six patients. The angle between the line perpendicular to the vertebral body and that from the vertebral body to the apex was defined as the “vertebral-apex angle (V-A angle).” The ratio of post- and preoperative angles, indicating the apex’s lateral rotation, was compared between patients with and without PVO. Results: The median (interquartile range) age and body weight at repair were 102 (79-176) days and 3.8 (2.6-4.8) kg, respectively. The 1-year survival rate was 83% (median follow-up, 29 [11-36] months). PVO occurred in seven patients (39%), who showed an obstruction of one or two branches in the apex side. The postoperative V-A angle (46° [45°-50°] vs. 36° [29°-38°], P = 0.001) and the ratio of post- and preoperative V-A angles (1.27 [1.24-1.42] vs. 1.03 [0.98-1.07], P = 0.001) were significantly higher in the PVO group than in the non-PVO group. The cut-off values of the postoperative V-A angle and ratio were 41° and 1.17, respectively. Conclusions: A postoperative rotation of the heart apex into the ipsilateral thorax was a risk factor for branch PVO after TAPVC repair.

scholarly journals Mid-term results of modified L-shaped incision technique for supracardiac total anomalous pulmonary venous connection

2020 ◽  
Vol 58 (6) ◽  
pp. 1261-1268
Author(s):  
Zicong Feng ◽  
Yang Yang ◽  
Fengpu He ◽  
Kunjing Pang ◽  
Kai Ma ◽  
...  
Keyword(s):  
Propensity Score ◽  
Multivariable Analysis ◽  
Pulmonary Venous Obstruction ◽  
Kaplan Meier ◽  
Incision Technique ◽  
Atrial Isomerism ◽  
Anomalous Pulmonary Venous Connection ◽  
Mid Term Results ◽  
Acceptable Outcome

Abstract OBJECTIVES Surgical outcomes of supracardiac total anomalous pulmonary venous connection (TAPVC) repair by the posterior technique (PT) remain unsatisfactory. This study aimed to compare the outcomes of the modified L-shaped incision technique with the PT for supracardiac TAPVC repair. METHODS From January 2009 to December 2019, 121 consecutive patients with supracardiac TAPVC undergoing surgical repair in our institution were included (L-group, n = 53; PT group, n = 68). A propensity score-matched analysis was performed. Patients with single-ventricle physiology or atrial isomerism were excluded. All clinical data were retrospectively analysed. RESULTS In the unmatched cohort, the median follow-up duration was 33 months (interquartile range 26–65 months). There were 5 operative mortalities (4.1%) and 12 late mortalities (9.9%). Postoperative pulmonary venous obstruction (PVO) was documented in 21 patients. After matching (52 pairs), the overall survival rate in the L-group was 88.2% at both 3 and 5 years. For the propensity score-matched patients with preoperative PVO (n = 20), statistically significant differences (P = 0.002) were found by Kaplan–Meier curves with freedom from death and postoperative PVO at 1 and 3 years of 100% and 85.7% [standard deviation (SD): 13.2%] in the L-group and 90% (SD: 9.5%) and 22.9% (SD: 14.1%) in the PT group, respectively. Multivariable analysis revealed that the use of the PT was an independent risk factor for death and postoperative PVO (hazard ratio 4.12, 95% confidence interval 1.12–15.16; P = 0.03). CONCLUSIONS The modified L-shaped incision technique provided an acceptable outcome for supracardiac TAPVC repair. Compared with PT, the modified L-shaped incision technique was significantly associated with decreased death and postoperative PVO in patients with obstructed supracardiac TAPVC.

Repair of a mixed form of supracardiac total anomalous pulmonary venous connection

2020 ◽  
pp. 1-3
Author(s):  
Yuki Kawasaki ◽  
John N. Dentel ◽  
Henry L. Walters ◽  
James M. Galas ◽  
Daisuke Kobayashi
Keyword(s):  
Superior Vena ◽  
Pulmonary Veins ◽  
Vena Cava ◽  
Right Atrial ◽  
Pulmonary Venous Obstruction ◽  
Mixed Form ◽  
Vertical Vein ◽  
Vena Cava Superior ◽  
Anomalous Pulmonary Venous Connection

Abstract Total anomalous pulmonary venous connection is a rare congenital heart defect. We report an infant with a mixed form of supracardiac TAPVC, in whom all pulmonary veins, except the right upper, entered a pulmonary venous confluence that is connected to a vertical vein and drained into the superior vena caval–right atrial junction. Several segmental right upper pulmonary veins entered the superior vena cava, superior to the entry of the vertical vein. Surgical repair consisted of the Warden procedure combined with direct anastomosis of the vertical vein to the left atrium. Separate pulmonary venous drainage pathways decreased the risk of post-operative pulmonary venous obstruction. Our patient had an uneventful post-operative course and encouraging 2-month follow-up echocardiography. Careful follow-up is warranted to detect post-operative complications, including obstruction of the pulmonary venous and cavoatrial anastomoses.

scholarly journals Antenatal diagnosis of total anomalous pulmonary venous connection in functional single ventricle hearts: Outcomes over 13-year period

Ultrasound ◽  
2018 ◽  
Vol 26 (1) ◽  
pp. 42-48
Author(s):  
Precylia Fernandes ◽  
Lito Mantagou ◽  
Ram Ramaraj ◽  
Umber Agarwal ◽  
Joyce Su Ling Lim
Keyword(s):  
Cardiac Surgery ◽  
Single Ventricle ◽  
Antenatal Diagnosis ◽  
Right Atrial ◽  
Cardiac Anomaly ◽  
Compassionate Care ◽  
Cardiac Ventricle ◽  
Fontan Surgery ◽  
Atrial Isomerism ◽  
Anomalous Pulmonary Venous Connection

Introduction A functionally single cardiac ventricle seen on foetal ultrasound scan carries a guarded prognosis. The antenatal diagnosis of anomalous pulmonary venous connection (APVC) remains challenging, if there is no associated structural cardiac abnormality. Antenatally, a combination of complex cardiac anomaly with suspected isomerism should raise the possibility of associated total anomalous pulmonary venous connection (TAPVC). There needs to be a high index of suspicion for TAPVC, in functional single ventricle and suspected isomerism, as this carries a very grim outcome postnatally. We illustrate foetal echocardiographic findings of suspected TAPVC and review outcomes of antenatal versus postnatal diagnosis of TAPVC with functional single ventricle. Methods We retrospectively reviewed our database over 13 years, focusing on foetal cardiac diagnosis, pregnancy outcomes, management and outcomes of livebirths with diagnosis of TAPVC with functional single ventricle. Results Thirteen patients were included in the review. For the nine antenatal patients, three pregnancies were terminated and six babies were born alive (four babies had compassionate care, two babies had cardiac surgery). One baby is alive at 8.5 years, after Fontan surgery. For the four postnatal patients, three babies had compassionate care (one alive at age 8.1 years) and one baby had cardiac surgery (died age nine weeks). Ten of the 13 patients have right atrial isomerism. Of these 10 patients, only two are alive. For the three non-isomeric babies, only one baby is still alive. There is heterogeneity of the type of TAPVC diagnosed with no particular group that offered better survival. Conclusion Antenatal diagnosis of TAPVC, even in the context of functional single ventricle remains challenging. If isomerism is suspected, targeted evaluation of pulmonary venous connection should be done. This combination of cardiac lesion carries a very grim outcome. The ability to make this diagnosis antenatally will add to the information and counselling given to these parents.

Early- and Middle-Term Surgical Outcomes in Patients with Heterotaxy Syndrome

Cardiology ◽  
2015 ◽  
Vol 133 (3) ◽  
pp. 141-146 ◽  
Author(s):  
Weidan Chen ◽  
Li Ma ◽  
Hujun Cui ◽  
Shengchun Yang ◽  
Yuansheng Xia ◽  
...  
Keyword(s):  
Fontan Procedure ◽  
Univariate Analysis ◽  
Survival Rates ◽  
Right Atrial ◽  
Pulmonary Venous Obstruction ◽  
Heterotaxy Syndrome ◽  
Middle Term ◽  
Palliative Procedure ◽  
Atrial Isomerism

Objectives: Heterotaxy syndrome is a recognized risk factor for surgical cardiac interventions. We evaluated the early- and middle-term results of a surgical intervention for patients with heterotaxy syndrome. Methods: A total of 42 patients with heterotaxy syndrome were enrolled (September 2008 to March 2015). Left and right atrial isomerism were identified in 26% (11 out of 42) and 74% of patients (31 out of 42), respectively. The median age of the patients at the time of surgery was 6.8 months (range: 5 days to 22.3 years). Biventricular repair was completed in 3 patients with left atrial isomerism. Seventeen out of 39 patients who were scheduled for single ventricular repair completed a modified Fontan procedure. Results: The hospital mortality rate was 4.7% (2 out of 42). Another 5 deaths occurred in the remaining survivors following hospital discharge with a follow-up duration of 45.8 ± 23.6 months (range: 13-111 months). The 1-year and 5-year survival rates were 88.1% (37/42) and 83.3% (35/42), respectively. Univariate analysis and multivariate analysis identified pulmonary venous obstruction and atrioventricular valve replacement as additional risk factors for mortality. Conclusions: Right ventricular bypass surgery remains the preferred palliative procedure for patients with heterotaxy syndrome. Based on the current results, the early- and middle-term outcomes are satisfactory.

Poor outcome for patients with totally anomalous pulmonary venous connection and functionally single ventricle

2009 ◽  
Vol 19 (6) ◽  
pp. 594-600 ◽  
Author(s):  
Nicodème Sinzobahamvya ◽  
Claudia Arenz ◽  
Julia Reckers ◽  
Joachim Photiadis ◽  
Peter Murin ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Right Ventricular ◽  
Pulmonary Atresia ◽  
Right Ventricular Outflow Tract ◽  
Right Atrial ◽  
Pulmonary Venous Obstruction ◽  
Actuarial Survival ◽  
Poor Outcome ◽  
The Right ◽  
Anomalous Pulmonary Venous Connection

AbstractTotally anomalous pulmonary venous connection, when also associated with a functionally univentricular connection, is known to have a poor outcome. We retrospectively analysed results for 19 patients undergoing surgery for this combination of lesions between 1995 and February 2009.Of the patients, 12 were neonates, with 11 presenting with signs of pulmonary venous obstruction. In 3 patients, a modified Blalock-Taussig shunt had been constructed. The dominant ventricle was of right ventricular morphology in 17 of the 19 patients, and double inlet was present in all bar 1. Pulmonary atresia or stenosis was found in 14 patients, a common atrioventricular junction in 14 patients, and isomerism of the right atrial appendages in 12 patients, respectively. Comprehensive Aristotle scores ranged from 14 to 23.50. The mean was 16.55, with a standard deviation of 2.19. Pulmonary venous rerouting was combined in 6 patients with construction of a modified Blalock-Taussig, in 4 with banding of the pulmonary trunk, in another 4 with a bidirectional Glenn anastomosis, in 3 with creation of a total cavo-pulmonary connection, and in 1 each with enlargement of the right ventricular outflow tract and the Norwood procedure. Of the cohort, 8 patients died early due to pulmonary hypertension, with all patients having Aristotle scores of at least 18 points dying. Among the 11 early survivors, 5 needed mechanical ventilation for over a week, but 5 patients died later, 3 due to pulmonary hypertension and 2 due to infection. Actuarial survival stabilized at 31.6%, with standard error of 10.7%, from one year onwards. Conversion to the Fontan circulation was successful in 5 patients.Our experience confirms that totally anomalous pulmonary venous connection, when associated with the functionally univentricular arrangement, carries one of the worst outcomes in current surgical practice. Use of the Aristotle comprehensive complexity scores effectively discriminates those patients with this condition at particularly high risk.

Sign in / Sign up

Export Citation Format

Share Document