Long-term outcome and complications of patients with single ventricle

Background: We have previously shown that neonates in profound cardiogenic shock due to severe Ebstein’s anomaly can be successfully salvaged with fenestrated right ventricular (RV) exclusion and systemic to pulmonary shunt (modified Starnes procedure). The long-term outcome of single ventricle management in these patients is not known. Methods: We retrospectively reviewed the records of 26 patients who underwent neonatal Starnes procedure between 1989 and 2011. Patient demographics, clinical variables and outcome data were collected. Data is presented as mean ± standard errors or median (interquartile ranges). Results: 26 patients (12, 46% boys) underwent Starnes procedure at 7 (5-9) days of life. All were intubated and on prostacyclin infusion, 24 (92%) were inotrope-dependent and 23 (88%) had no antegrade flow from the RV. Two patients had had prior intervention (one tricuspid annuloplasty and one shunt alone). Three patients underwent non-fenestrated RV exclusion, two (67%) of whom died. Of the remaining 23, 3 (13%) died during the same hospitalization. The 21 neonatal survivors have been followed for 7 (6-8) years. One patient died after Glenn. The remaining 20 have successfully undergone Fontan completion with an indexed pulmonary resistance of 1.8 (1.2-2.3) W/m2 and mean pulmonary pressure of 12 (9-18) mm Hg. At last follow-up, all patients have normal left ventricular function, and all but one patient are in NYHA Class I symptoms. Two patients have required pacemaker implantation, while the rest are in sinus rhythm. Survival at 1, 5 and 10 years are 81±4%, 77±3% and 77±3%, respectively. Conclusion: Long-term single ventricle outcomes amongst neonatal survivors of modified Starnes procedure are excellent. There is reliable remodeling of the excluded RV and excellent function of the left ventricle.

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Long-Term Outcome of Direct Relief of Subaortic Stenosis in Single Ventricle Patients

World Journal for Pediatric and Congenital Heart Surgery ◽

10.1177/2150135118793087 ◽

2018 ◽

Vol 9 (6) ◽

pp. 638-644 ◽

Cited By ~ 3

Author(s):

Friso M. Rijnberg ◽

Vladimir Sojak ◽

Nico A. Blom ◽

Mark G. Hazekamp

Keyword(s):

Heart Block ◽

Single Ventricle ◽

Ventricular Dysfunction ◽

Simple Procedure ◽

Direct Approach ◽

Subaortic Stenosis ◽

Term Outcome ◽

Long Term Outcome ◽

Pseudo Aneurysm

Background: Single ventricle patients with unrestrictive pulmonary blood flow and (potential) subaortic stenosis are challenging to manage and optimal surgical strategy is unknown. Direct relief of subaortic stenosis by enlargement of the ventricular septal defect and/or subaortic chamber has generally been replaced by a Damus-Kaye-Stansel or Norwood procedure due to concerns of iatrogenic heart block, reobstruction, or ventricular dysfunction. Studies reporting long-term outcome after the direct approach are limited. The aim of our study was to describe and analyze our experience with direct relief of subaortic stenosis in single ventricle patients. Methods: Demographic data, characteristics, and pre-operative, operative and outcome details were collected for children undergoing direct relief of subaortic stenosis between 1989 and 2016. Results: Twenty-three patients (median age: 7.4 months, range: 10 days to 5.5 years) underwent direct relief of subaortic stenosis. Complete follow-up was available for all patients (median: 15.6 years, range: 34 days to 26.3 years). Seven (30%) patients had recurrence of subaortic stenosis. One (4%) patient developed complete heart block and one patient developed moderate ventricular dysfunction. Five (50%) patients developed a (pseudo)aneurysm at site of the patch and ventriculotomy. There were two perioperative deaths. Eighty-six percent of patients underwent a successful Fontan procedure. Conclusions: Direct relief of subaortic stenosis is associated with a substantial risk of reobstruction and patch (pseudo)aneurysm formation. However, risk of heart block is low and long-term outcome is good with the majority of patients reaching Fontan completion. In our opinion, the direct approach appears to be a good and relatively simple procedure in selected cases for the treatment of subaortic stenosis.

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Pregnancy and Delivery in Functional Single Ventricle Patient; Successful Long-Term Outcome after Right Ventricle Exclusion and Fontan Operation

Korean Circulation Journal ◽

10.4070/kcj.2016.46.1.111 ◽

2016 ◽

Vol 46 (1) ◽

pp. 111 ◽

Cited By ~ 2

Author(s):

Sang Yoon Kim ◽

Sungkyu Cho ◽

Eunseok Choi ◽

Woong-Han Kim

Keyword(s):

Right Ventricle ◽

Single Ventricle ◽

Fontan Operation ◽

Term Outcome ◽

Long Term Outcome ◽

Pregnancy And Delivery

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Long term outcome of single ventricle physiology with pulmonary restriction not undergoing Fontan repair

European Heart Journal ◽

10.1093/ehjci/ehaa946.2205 ◽

2020 ◽

Vol 41 (Supplement_2) ◽

Author(s):

F Buendia ◽

B Ramirez ◽

P Gallego ◽

J.M Oliver ◽

S Montserrat ◽

...

Keyword(s):

Single Ventricle ◽

Term Outcome ◽

Long Term Outcome ◽

Single Ventricle Physiology ◽

Pulmonary Flow ◽

Adult Congenital ◽

Group 3 ◽

Group 2

Abstract Background Patients with univentricular physiology who do not complete the palliation to Fontan are a heterogeneous group with unknown long term outcome. Aims This study aimed at describing the clinical course and long-term survival of patients with SV physiology with restricted pulmonary flow that had not undergone a Fontan type of repair. Methods From the prospectively maintained databases of the adult congenital cardiac units of five tertiary referral centers, data from all SV physiology patients were obtained. Patients completing a Fontan type palliation or developing Eisenmenger physiology and segmental pulmonary hypertension were excluded. Baseline data were recorded on the first visit at adult congenital heart disease (ACHD) unit. The primary end point was death. Results 101 patients (50.5% females) were identified. Mean age at end of follow up was 39.3±11.3 years. Of these, 45 (44.6%) were unoperated (group 1, restricted forward pulmonary flow with or without pulmonary banding), 38 (37.6%) had undergone a cavopulmonary shunt as a definitive palliation (group 2) and 18 (17.8%) had aortopulmonary shunts (group 3). The main diagnosis was double inlet left ventricle (DILV) (N: 52, 51.5%) and most of the ventricle was left (82.2%). The principal reason for not performing a Fontan repair was mean pulmonary artery pressure >18 mmHg. At initial visit at the ACHD unit patients were 32.2±11.1 years of age. 35% of the patients were in NYHA class III-IV, with no differences between groups. However, patients in group 2 had worse oxygen saturation (p=002) and higher haemoglobin (p=0.037). After a mean follow-up of 7.3±4.1 years, mortality was 20.8% (21 patients), being sudden death (7p, 6.9%) the most frequent cause. Patients in group 3 showed worse ventricular function (p=0.0001) and a trend to higher mortality that did not reach statistical significance (HR 2.7, CI 95% 0.91–8.14, P=0.07). Conclusions Patients with single ventricle physiology not undergoing Fontan repair are a population of high risk, with sudden death as main driver of mortality. Patients palliated with aortopulmonary shunts are prone to worse ventricular function and a trend to higher mortality. Funding Acknowledgement Type of funding source: Public hospital(s)

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