Follow-up after biventricular repair of the hypoplastic left heart complex

Abstract OBJECTIVES In hypoplastic left heart complex patients, biventricular repair is preferred over staged-single ventricle palliation; however, there are too few studies to support either strategy. Therefore, we retrospectively characterized our patient cohort with hypoplastic left heart complex after biventricular repair to measure left-sided heart structures and assess our treatment strategy. METHODS Patients with hypoplastic left heart complex who had biventricular repair between 2004 and 2018 were retrospectively reviewed. Operative results were evaluated and echocardiographic mitral valve (MV) and aortic valve (AoV) dimensions, left ventricular length and left ventricular internal diastolic diameter (LVIDd) were measured preoperatively and during follow-up after 0.5, 1, 3, 5 and 10 years. RESULTS In 32 patients, the median age at surgery was 10 (interquartile range 5.0) days. The median follow-up was 6.19 (interquartile range 6.04) years. During the 10-year follow-up, the mean Z-scores increased from −2.82 to −1.49 and from −2.29 to 0.62 for MV and AoV, respectively. Analysis of variance results with post hoc paired t-tests showed that growth of left-sided heart structures was accelerated in the first year after repair, but was not equal, with the MV lagging behind the AoV (P = 0.033), resulting in significantly smaller MV Z-scores compared with AoV Z-scores at 10-year follow-up (P < 0.001). There were 2 (6%) early deaths. The major adverse events occurred in 4 (13%) patients. The surgical or catheter-based reintervention was required in 14 (44%) patients. CONCLUSIONS The growth rate of heart structures was most prominent during the first year after biventricular repair with lower growth rate of the MV compared with the AoV.

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Biventricular repair in the management of hypoplastic left heart syndrome

Cardiology in the Young ◽

10.1017/s1047951104006390 ◽

2004 ◽

Vol 14 (S1) ◽

pp. 101-104 ◽

Cited By ~ 4

Author(s):

Victor O. Morell ◽

James A. Quintessenza ◽

Jeffrey P. Jacobs

Keyword(s):

Aortic Arch ◽

Hypoplastic Left Heart Syndrome ◽

International Committee ◽

Left Ventricular ◽

Left Heart ◽

Hypoplastic Left Heart ◽

Biventricular Repair ◽

Cardiac Anomalies ◽

Left Heart Syndrome ◽

Hypoplastic Left Heart Complex

Hypoplastic left heart syndrome is the term introduced by Noonan and Nadas1to describe a spectrum of cardiac anomalies characterized by varying degrees of significant underdevelopment of the left heart and aortic arch. These cardiac anomalies include mitral valvar disease, left ventricular hypoplasia, aortic stenosis at subvalvar, valvar, and supravalvar levels, hypoplasia of the ascending aorta and aortic arch, and aortic coarctation. Although descriptive, hypoplastic left heart syndrome suffers from being a very unspecific term, since it encompasses multiple degrees and combinations of abnormalities involving the left sided structures. In an attempt to provide gradings of severity, Kirklin and Barratt-Boyes2categorized these patients falling into the syndrome into four classes, according to whether obstruction was found at one, two, or more levels, or whether there is aortic atresia. Then, in 1998, Tchervenkov and colleagues3introduced the term hypoplastic left heart complex to describe a set of patients falling within the spectrum of hypoplasia of the left heart, but in the absence of intrinsic aortic or mitral valvar stenosis, this concept subsequently being endorsed by the International Committee established by the Society of Thoracic Surgeons, together with the European Association of Cardiothoracic Surgery, to rationalize the approach to nomenclature and databases.4The analysis offered by Tchervenkov et al.3showed that, in certain circumstances, there are potentially patients considered to have hypoplasia of the left heart who might be candidates for biventricular repair. If this is the case, then it is important to establish how this subset can be recognized, and how they are best treated.

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Midterm Follow-Up After Biventricular Repair of the Hypoplastic Left Heart Complex

The Annals of Thoracic Surgery ◽

10.1016/j.athoracsur.2015.02.030 ◽

2015 ◽

Vol 99 (6) ◽

pp. 2150-2156 ◽

Cited By ~ 7

Author(s):

Jan Erik Freund ◽

Martijn H.T. den Dekker ◽

A. Christian Blank ◽

Felix Haas ◽

Matthias W. Freund

Keyword(s):

Left Heart ◽

Hypoplastic Left Heart ◽

Biventricular Repair ◽

Hypoplastic Left Heart Complex

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Growth of hypoplastic mitral valves in hypoplastic left heart complex and similar constellations after anatomical left superior vena cava correction

European Journal of Cardio-Thoracic Surgery ◽

10.1093/ejcts/ezaa286 ◽

2020 ◽

Vol 59 (1) ◽

pp. 236-243

Author(s):

Robert A Cesnjevar ◽

Frank Harig ◽

Moritz Dietz ◽

Muhannad Alkassar ◽

Wolfgang Waellisch ◽

...

Keyword(s):

Superior Vena Cava ◽

Coronary Sinus ◽

Superior Vena ◽

Vena Cava ◽

Innominate Vein ◽

Left Heart ◽

Hypoplastic Left Heart ◽

Mitral Inflow ◽

Z Scores ◽

Hypoplastic Left Heart Complex

Abstract OBJECTIVES Left superior vena cava (LSVC)-related obstruction of mitral inflow is a rare finding in patients with complex cardiac anomalies like hypoplastic left heart complex. We report our experience by establishing a left superior to right superior caval vein continuity (innominate vein creation by direct LSVC–right superior vena cava end-to-side-anastomosis), and coronary sinus unroofing if indicated for LSVC-related mitral inflow obstruction. METHODS Nineteen patients (median age: 1.0 ± 0.3 years; range: 7 days–4.8 years) underwent anatomical correction of LSVC without the use of foreign material in conjunction with repair or palliation of congenital anomalies in a single centre between April 2015 and November 2019. Indications for the procedure were LSVC-related obstruction of left ventricular inflow due to a dilated coronary sinus. Additional procedures included mitral (n = 7) or atrioventricular (n = 3) valve surgery, right ventricular to pulmonary artery conduit (n = 3), first stage palliation (n = 3) or biventricular repair (n = 5) of hypoplastic left heart complex. Three patients needed secondary mitral valve replacement (n = 3). RESULTS All LSVC or coronary sinus-related obstructions were effectively relieved. No patient died early, 2 patients died late after the procedure. One patient needed stenting of the superior vena cava below the unobstructed cephalad vein anastomosis at the former right superior vena cava-cannulation-site. Follow-up was complete and demonstrated an 89.5% survival after 2.5 ± 0.4 years. Innominate vein patency was 100% documented by echocardiography (n = 19), cardiac catheterization (n = 6) or both. Mean mitral valve z-scores before the operation were −1.7 ± 0.2 (range −3.8 to 0.3) and increased to 0.7 ± 0.2 (range −0.7 to 1.9) after LSVC repair. CONCLUSIONS Anatomical correction by surgical creation of an innominate vein is an effective method to relieve LSVC-related obstructions and promotes mitral valvar growth. Mitral ring sizes were at least normalized after surgery at the time of discharge. Further prospective follow-up studies to evaluate the growth potential of left-sided heart structures by reporting cardiac z-scores are needed to evaluate the true impact of coronary sinus unroofing.

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Erratum to: Development of an Echocardiographic Scoring System to Predict Biventricular Repair in Neonatal Hypoplastic Left Heart Complex

Pediatric Cardiology ◽

10.1007/s00246-014-1043-y ◽

2014 ◽

Vol 35 (8) ◽

pp. 1467-1468

Author(s):

Christopher Robin Mart ◽

Aaron Wesley Eckhauser

Keyword(s):

Scoring System ◽

Left Heart ◽

Hypoplastic Left Heart ◽

Biventricular Repair ◽

Hypoplastic Left Heart Complex

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Biventricular repair in patients with hypoplastic left heart complex

The Thoracic and Cardiovascular Surgeon ◽

10.1055/s-2007-967266 ◽

2007 ◽

Vol 55 (S 1) ◽

Author(s):

S Daebritz ◽

J Sachweh ◽

A Tiete ◽

R Sodian ◽

D Rassoulian ◽

...

Keyword(s):

Left Heart ◽

Hypoplastic Left Heart ◽

Biventricular Repair ◽

Hypoplastic Left Heart Complex

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Development of an Echocardiographic Scoring System to Predict Biventricular Repair in Neonatal Hypoplastic Left Heart Complex

Pediatric Cardiology ◽

10.1007/s00246-014-1009-0 ◽

2014 ◽

Vol 35 (8) ◽

pp. 1456-1466 ◽

Cited By ~ 10

Author(s):

Christopher Robin Mart ◽

Aaron Wesley Eckhauser

Keyword(s):

Scoring System ◽

Left Heart ◽

Hypoplastic Left Heart ◽

Biventricular Repair ◽

Hypoplastic Left Heart Complex

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Comparison of self-expandable and balloon-expanding stents for hybrid ductal stenting in hypoplastic left heart complex

Cardiology in the Young ◽

10.1017/s1047951116001347 ◽

2016 ◽

Vol 27 (5) ◽

pp. 837-845 ◽

Cited By ~ 2

Author(s):

Sebastian Goreczny ◽

Shakeel A. Qureshi ◽

Eric Rosenthal ◽

Thomas Krasemann ◽

Mohamed S. Nassar ◽

...

Keyword(s):

Hypoplastic Left Heart Syndrome ◽

Ductus Arteriosus ◽

Left Heart ◽

Stent Group ◽

Hypoplastic Left Heart ◽

Group 4 ◽

Left Heart Syndrome ◽

Term Performance ◽

Hypoplastic Left Heart Complex

AbstractObjectivesWe aimed to compare the procedural and mid-term performance of a specifically designed self-expanding stent with balloon-expandable stents in patients undergoing hybrid palliation for hypoplastic left heart syndrome and its variants.BackgroundThe lack of specifically designed stents has led to off-label use of coronary, biliary, or peripheral stents in the neonatal ductus arteriosus. Recently, a self-expanding stent, specifically designed for use in hypoplastic left heart syndrome, has become available.MethodsWe carried out a retrospective cohort comparison of 69 neonates who underwent hybrid ductal stenting with balloon-expandable and self-expanding stents from December, 2005 to July, 2014.ResultsIn total, 43 balloon-expandable stents were implanted in 41 neonates and more recently 47 self-expanding stents in 28 neonates. In the balloon-expandable stents group, stent-related complications occurred in nine patients (22%), compared with one patient in the self-expanding stent group (4%). During follow-up, percutaneous re-intervention related to the ductal stent was performed in five patients (17%) in the balloon-expandable stent group and seven patients (28%) in self-expanding stents group.ConclusionsHybrid ductal stenting with self-expanding stents produced favourable results when compared with the results obtained with balloon-expandable stents. Immediate additional interventions and follow-up re-interventions were similar in both groups with complications more common in those with balloon-expandable stents.

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