Borderline Hypoplastic Left Heart Malformations: Norwood Palliation or Two-Ventricle Repair?

Daebritz, Sabine; Tiete, A.; Rassoulian, D.; Roemer, U.; Kozlik-Feldmann, R.; Sachweh, Joerg S.; Netz, Heinrich; Reichart, B.

doi:10.1055/s-2002-34580

Cited by 14 publications

(9 citation statements)

References 16 publications

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“…Complete elimination of the left-to-right shunt by closing the atrial septal defect (ASD) has been suggested to reduce the risk of low cardiac output due to excessive left-to-right shunt through an unrestrictive communication. Improvement of left ventricular filling and therefore subsequent left ventricular growth is expected [ 1 , 10 , 11 ]. On the other hand, the sudden increase of preload may result in left ventricular overload and distension determining severe left ventricular dysfunction with sometimes a “stormy” early postoperative period [ 1 , 9 , 10 ].…”

Section: Introductionmentioning

confidence: 99%

“…On the other hand, the sudden increase of preload may result in left ventricular overload and distension determining severe left ventricular dysfunction with sometimes a “stormy” early postoperative period [ 1 , 9 , 10 ]. An alternative surgical option as a compromise between both approaches is the partial ASD-closure with a fenestrated patch [ 10 , 11 ].…”

Section: Introductionmentioning

confidence: 99%

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Aortic arch obstruction neonates with biventricular physiology: left-open compared to closed inter-atrial communication during primary repair – a retrospective study

Rüffer

Bechtold

Purbojo

et al. 2015

J Cardiothorac Surg

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BackgroundLeaving an inter-atrial communication (IAC) open for left atrial decompression is often recommended in neonates with aortic arch obstruction undergoing primary repair. In this study, outcomes in these patients were compared to those with intact atrial septum after repair.MethodsBetween 2000 and 2013, 53 consecutive neonates with severe aortic arch obstruction (hypoplasia: n = 45, interruption: n = 8) underwent primary repair from an anterior approach. Median age and weight were 8 days (range: 2–30) and 3.2 kg (range: 2.4-4.4), respectively. Cardiac morphology included a ventricular septal defect (VSD, large: n = 28, small: n = 7), malposition of great arteries (n = 10), and severe left ventricular outflow tract obstruction (LVOTO, n = 10). During corrective surgery IAC was closed (group-I, n = 37) or partially left-open (group-II, n = 16). Primary endpoints were hospital death, and re-intervention (surgery and/or balloon) due to aortic arch re-coarctation or recurrent LVOTO. Statistically significant variables by univariate analysis were incorporated in the corresponding multivariable regression model.ResultsRegarding morphological discrepancies more patients in group-II presented with LVOTO (p = 0.05), or the combination of arch hypoplasia, intact ventricular septum and normal ventriculo-arterial connection (p = 0.017). Hospital mortality was 8.1% in group-I and 37.5% in group-II (p = 0.016). Re-intervention was performed in 13 patients (group-I: n = 6 vs. group-II: n = 7) due to aortic arch re-coarctation (n = 12) and/or recurrent LVOTO (n = 3), and resulted in a Kaplan-Meier freedom from re-intervention of 87 ± 6% and 79 ± 8% in group-I, and 64 ± 14% and 64 ± 14% in group-II after 1 and 5 years, respectively (p = 0.016). Multivariate analysis revealed LVOTO as an independent risk factor for hospital death (p = 0.042), whereas both LVOTO and left-open IAC (p = 0.001 and 0.01) were independent risk factors for re-intervention.ConclusionsA left-open IAC increases risk of re-intervention at the left heart aorta complex. Sustained left-to-right shunting on atrial level seems to induce preload reduction of the often restrictive left ventricle leading to decreased aortic blood flow.

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Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Aortic arch obstruction neonates with biventricular physiology: left-open compared to closed inter-atrial communication during primary repair – a retrospective study

Rüffer

Bechtold

Purbojo

et al. 2015

J Cardiothorac Surg

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“…The growth of the left ventricle in patients with hypoplastic left heart complex has been proved after biventricular type of repair, provided the accomplishment of complete relief of the systemic obstruction [35][36][37][38][39]. The preferred surgical technique is the reconstruction of the ascending aorta and aortic arch using either a pulmonary autograft or homograft.…”

Section: Hypoplastic Left Heart Complexmentioning

confidence: 99%

“…Since the neonatal left ventricle is already functioning at the plateau level of the Starling's curve, any volume overload can determine severe left ventricular dysfunction, and eventually death [38]. An alternative surgical option is the partial closure of the atrial septal defect: a fenestration allows a substantial reduction of the excessive left-to-right shunt in order to improve the left ventricular filling, and in the same time leaves enough time to the left ventricle to adapt to the new loading conditions [38,39].…”

Section: Hypoplastic Left Heart Complexmentioning

confidence: 99%

Borderline left ventricle

Corno

2005

European Journal of Cardio-Thoracic Surgery

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The first problem to solve when dealing with the topic 'borderline left ventricle' is to find the appropriate definition. Several parameters have been taken into consideration, either morphometric (diameter of the mitral valve, indexed mitral valve area, left ventricular inflow dimension, left ventricular cross-sectional area, ratio between the apex-to-base left ventricular dimension and right ventricular dimension, left ventricular long axis to heart long axis ratio, left ventricular end diastolic volume, left ventricular mass index, ratio of the right/left ventricular wall thickness, presence of endocardial fibroelastosis, cardiac apex not formed by the left ventricle, diameter of the ventriculo-aortic junction, diameter of the aortic valve annulus and indexed aortic root diameter) as well as functional (left ventricular ejection fraction, left ventricular end diastolic pressure, mean pulmonary artery pressure, direction of the blood flow in the ascending aorta and at the level of the patent ductus arteriosus). Pre-operative determination whether the left ventricle is adequate to sustain the systemic circulation, or it may became adequate with the available surgical approaches, and therefore a bi-ventricular type of repair is feasible, can be extremely difficult, particularly in the presence of a 'borderline left ventricle'. In the clinical practice pediatric cardiologists and cardiac surgeons are faced with the problem of the 'borderline left ventricle' in four different groups of congenital heart defects: (a) aortic valve stenosis, (b) aortic coarctation, with or without hypoplastic aortic arch, (c) hypoplastic left heart complex, (d) right ventricular pressure and/or volume overload. In all the above situations in the presence of a left ventricle smaller than normal a very exhaustive approach has been reviewed in the decision making process, taking in account the literature reports as well as the personal experience. In each patient with 'borderline left ventricle' the elements to be considered for the decision making process between uni- and bi-ventricular type of repair, or for less ideal options of management, are the following: morphometric and functional parameters, hemodynamic data, available surgical options, results of the personal and institutional experience.

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“…The difficult decision about univentricular as opposed to biventricular repair may be aided by fetal observations. 3,4 A vaginal delivery is preferable for infants with hypoplastic left heart syndrome, and avoids unnecessary risk for the mother as long as the fetus has no signs of cardiac failure. We use the cardiovascular profile score to select those fetuses requiring special attention, and we have shown that this cardiovascular score correlates with the Tei index.…”

Section: Prenatal Assessmentmentioning

confidence: 99%

Neonatal hemodynamics in patients with hypoplastic left heart syndrome

Huhta

2004

Cardiol Young

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The neonate with hypoplastic left heart syndrome presents a challenge for clinical diagnosis and management. Three diagnostic goals must be met. First, it is necessary to make an etiologic cardiac diagnosis so as to rule out any genetic abnormality. Second, the anatomic cardiac diagnosis is made by segmental echocardiographic analysis, including details of the atrial arrangement, venous return, the patency of the arterial duct, atrial anatomy, and the arrangement of the aortic arch. Finally, the physiologic cardiac diagnosis is made by Doppler evaluation. In some patients, the diagnosis of hypoplastic left heart syndrome is not synonymous with functionally univentricular physiology, and a bi-ventricular repair can be achieved.1

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Borderline Hypoplastic Left Heart Malformations: Norwood Palliation or Two-Ventricle Repair?

Abstract: Some anatomical subsets of HLHS with borderline mitral valves and small left ventricles may undergo two-ventricle repair despite severe LVOTO. Mortality and morbidity seem to be lower, but selection criteria are so far not defined.

Cited by 14 publications

References 16 publications

Aortic arch obstruction neonates with biventricular physiology: left-open compared to closed inter-atrial communication during primary repair – a retrospective study

Aortic arch obstruction neonates with biventricular physiology: left-open compared to closed inter-atrial communication during primary repair – a retrospective study

Borderline left ventricle

Neonatal hemodynamics in patients with hypoplastic left heart syndrome

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