Left ventricular growth in selected hypoplastic left ventricles: outcome after repair of coarctation of aorta

doi:10.1016/S0003-4975(99)00621-9

The Annals of Thoracic Surgery

Volume 68, Issue 2, August 1999, Pages 549-555

https://doi.org/10.1016/S0003-4975(99)00621-9 Get rights and content

Abstract

Background. Models that predict survival in neonates with left ventricular hypoplasia and critical aortic stenosis may not be applicable to neonates with left ventricular hypoplasia and coarctation.

Methods and Results. We report 8 infants with severe aortic coarctation and left ventricular hypoplasia. Mean age was 18 days (range 1–48 days), and mean weight was 3.5 kg (range 2.7–4.3 kg). Associated diagnoses included mild aortic stenosis (4), ventricular septal defect (2), and venous anomalies (2). All had coarctation repair as a primary procedure (3 of these had concomitant intracardiac procedures); 7 had subsequent operations. All are alive and well 1.1–6.7 years (mean 3.1 years) after the first surgery. Progressive increases were observed in aortic and mitral diameters, and in left ventricular dimensions, areas, and volumes when the preoperative, earliest postoperative, and most recent echocardiograms were compared.

Conclusions. Despite severe left ventricular hypoplasia, a two-ventricle repair is possible in selected cases. The prognostic criteria for left ventricular hypoplasia in critical aortic stenosis may not be applicable to infant coarctation. Relief of coarctation may result in the growth of the very small left ventricle, especially when the aortic root and mitral diameters are satisfactory.

Section snippets

Patients and methods

We reviewed the medical records and echocardiographic studies of all infants treated at the Children’s Memorial Hospital in Chicago during the period 1989–1995, with an echocardiographic diagnosis of severe CoA and LV hypoplasia, who required surgical intervention by the age of 6 months. Exclusion criteria were significant aortic valve stenosis (peak Doppler gradient ≥ 36 mm Hg), aortic atresia, mitral atresia, transposition of great arteries, and common atrioventricular canal.

Results

Of a total of 72 cases reviewed, 8 infants (6 boys, 2 girls) satisfied the selection criteria. Mean age at diagnosis was 18 days (range 1–48 days), whereas the mean weight was 3.5 kg (range 2.7–4.3 kg). Presenting signs and symptoms included low cardiac output (6 patients), cyanosis (1 patient), and heart murmur (1 patient). All had associated structural cardiac anomalies (Table 1), with 4 having doppler evidence of mild valvar AS. Seven of 8 had either an atrial septal defect or patent foramen

Comment

For certain heart defects, concern about adequacy of LV size to sustain the systemic circulation has been controversial 3, 9, 10, 11. In the presence of mitral or aortic atresia, a two-ventricle repair is not feasible. Depending on institutional experience, a sequence of palliation culminating in a modified Fontan procedure, cardiac transplantation, or no treatment may be chosen. In right ventricular volume overload, as seen in total anomalous pulmonary venous connection, marked hypoplasia of

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Cited by (32)

Midterm follow-up after biventricular repair of the hypoplastic left heart complex
2015, Annals of Thoracic Surgery
In neonates with hypoplastic left heart complex (HLHC), biventricular repair is considered superior to univentricular repair. The Z-scores of the mitral and the aortic valve annulus are primary factors for the choice of repair. Predictive cutoff values for the feasibility and optimal outcome of biventricular repair are unknown. This study assesses the growth of left side heart structures and the midterm outcome after biventricular repair with an interatrial fenestration in our HLHC population.
Retrospective study of 19 HLHC patients who underwent biventricular repair in a single tertiary referral center between 2004 and 2013. The cardiac dimensions (mitral and aortic valve annulus, left ventricle inlet length, left ventricular internal diastolic dimension) were measured before and at 6, 12, 24, and 48 months after biventricular repair.
The follow-up ranged from 2 to 98 months. There was no early mortality, and the midterm survival rate was 95%. One patient died of a noncardiac- and nonintervention-related cause. Seven patients (37%) required a total of 8 reinterventions because of recurring or residual obstructive lesions. After biventricular repair, the left cardiac structures grew significantly.
Neonatal biventricular repair is successful and safe in HLHC patients, even with preoperative mitral and aortic valve annulus Z-scores of −4.5 and −5.5, respectively. Residual or restenosis required reintervention in 37% of our HLHC population, but was not significantly correlated with the magnitude of the preoperative Z-scores. Within the first 6 months of follow-up, the Z-scores almost normalized.
Comparability of Z-score equations of cardiac structures in hypoplastic left heart complex
2013, Journal of the American Society of Echocardiography
Citation Excerpt :
They preclude comparison of series. Alboliras et al.13 investigated the length of the left ventricle and the short-axis diameter of the left ventricle in hypoplasia of the left ventricle in patients with severe coarctation. They concluded that relief of the coarctation results in the growth of the very small left ventricle, particularly when the aortic and mitral diameters are satisfactory, and the LV length is 80% of the right ventricular length.
Hypoplastic left heart complex (HLHC) is characterized by a mitral valve or an aortic valve annular Z score < −2, antegrade flow in the ascending aorta, ductal dependency, coarctation or aortic arch hypoplasia, and absence of significant (sub)valvar stenosis. The Z scores of the mitral and aortic valve annuli are major determinants of HLHC. Therefore, the algorithm for Z-score calculation is essential for diagnosis. However, no single universal method of calculation is in use. In the scientific literature addressing HLHC, various Z-score calculation methods have been applied. The aim of this study was to evaluate Z scores derived from two-dimensional echocardiographic dimensions in patients with HLHC.
To compare the different published methods using two-dimensional echocardiographic measures for Z-score calculation, a cohort of 18 newborns diagnosed with HLHC was retrospectively evaluated. In addition, the methods to determine body surface area in newborns were evaluated.
Three Z-score calculation methods were included and compared. Using the method of Daubeney et al. to calculate Z scores in our cohort illustrated a lack of correlation beyond a Z score < 0, compared with the methods of Zilberman et al. and Pettersen et al. Z scores calculated using Zilberman et al.'s and Pettersen et al.'s methods were fairly consistent. The equations used by Pettersen et al. are based on the largest population of neonates.
Although the different methods for calculating Z scores for mitral and aortic valve dimensions correspond fairly well in the normal range, Z scores < −2 diverge substantially. A useful scientific comparison of published data and outcomes of patients with HLHC remains elusive. The Z-score calculation algorithms used by Pettersen et al. appear to be the most appropriate for use in an evaluation of HLHC. Because these different methods can yield different values, reporting the method as well as the Z score is essential for an accurate diagnosis. Similarly, the method used to determine body surface area should be reported.
Long-term follow-up of neonatal coarctation and left-sided cardiac hypoplasia
2013, American Journal of Cardiology
Citation Excerpt :
Furthermore, none of our patients underwent reintervention for LV inflow or outflow obstruction after intermediate follow-up. Although several investigators have shown more encouraging short-term outcomes after neonatal CoA repair in the setting of left-sided cardiac hypoplasia,5–7,15 concerns remain that longstanding LV diastolic dysfunction might lead to the late development of pulmonary hypertension from chronic elevation of LV filling pressures and pulmonary venous hypertension.16 We were able to show that with ≥15 years of follow-up, all but 1 patient with measured tissue Doppler indexes had normal filling pressures according to an E/e′ ratio of <12.17
Neonatal coarctation of the aorta (CoA) is often associated with hypoplastic left-sided cardiac structures. Limited data are available regarding the long-term clinical outcomes. Our purposes were to assess the following in neonates with CoA and left-sided cardiac hypoplasia: (1) left-sided cardiac structure growth over time, (2) echocardiographic parameters predicting reintervention, and (3) long-term outcomes and reintervention rates. Data were collected for all patients born with isolated CoA, along with a mitral or aortic Z-score of <−2, who underwent repair before 2 months of age from January 1993 to April 1997. Recent follow-up data were available for 51 of 63 patients (81%) aged 15.4 ± 1.5 years (range 11 to 18). Of the 51 patients, 46 (90%) had a recent echocardiogram, all with normal left ventricular systolic function. The mitral and aortic valve annulus Z-scores increased significantly from the initial measurements: −3.29 ± 1.54 to −0.94 ± 0.58 (p <0.0001) and −3.25 ± 1.98 to 0.08 ± 1.81 (p <0.0001). No significant change was seen from intermediate (6.1 ± 1.6 years) to the latest follow-up examination. Of the 51 patients, 12 (24%) required reintervention at 3 ± 4.5 years; 9 for repeat CoA, 4 for subaortic stenosis, and 2 for aortic stenosis (some in combination). The initial echocardiographic parameters were not associated with reintervention. The overall freedom from reintervention was 76% at 15 years of follow-up. In conclusion, the long-term outcomes after isolated CoA repair with associated left-sided cardiac hypoplasia were excellent. Both aortic and mitral valve sizes had increased substantially by intermediate follow-up but tended to normalize afterward. Although 24% of the patients required reintervention, significant left ventricular inflow or outflow tract obstruction was uncommon.
Borderline hypoplasia of the left ventricle in neonates: Insights for decision-making from functional assessment with magnetic resonance imaging
2008, Journal of Thoracic and Cardiovascular Surgery
Citation Excerpt :
Predictors of early postoperative outcome must address whether the LV, after BV repair, is able to generate enough cardiac output to sustain the systemic circulation. Nearly all published predictors, however, are based on 2-dimensional anatomic rather than functional, indices.13-16 At our institution, there are two indications for cardiac catheterization in patients with marginally small LVs: One is to outline anatomy that cannot be detailed by echocardiography (or MRI), which was not the case in any of the patients presented here.
We sought to compare the usefulness of echocardiography and magnetic resonance imaging in neonates with a borderline small left ventricle.
The preoperative magnetic resonance and echocardiography studies of 20 consecutive patients (mean age 10 ± 9 days) undergoing magnetic resonance imaging were analyzed. The diagnoses were aortic stenosis (n = 3), hypoplastic left heart complex (n = 12), and unbalanced atrioventricular septal defect (n = 5). The magnetic resonance imaging protocol included ventricular volumetry, flow measurements, and angiography. Potential left ventricular volumes, assuming an ideal geometric shape, were calculated by mathematically “unfolding” the compressed left ventricle.
Left ventricular end-diastolic volume was 16.0 ± 7.0 mL/m² of body surface area by echocardiography and 33.5 ± 15.5 mL/m² by magnetic resonance imaging. Echocardiography consistently underestimated left ventricular volume and did not correlate with magnetic resonance. Of all echocardiographic parameters, mitral valve z-score was the best predictor of left ventricular end-diastolic volume by magnetic resonance (r = 0.77; P = .02). The average potential volume increase was 8.8% for aortic stenosis, 35.0% for atrioventricular septal defect and 23.0% for hypoplastic left heart complex patients. Aortic valve diameter did not correlate with flow volume in the ascending aorta. Sixteen (80%) of 20 patients underwent biventricular repair, without early mortality. Of these, only 5 (31.3%) had a preoperative left ventricular end-diastolic volume of more than 20 mL/m² by echocardiography.
Magnetic resonance imaging is feasible in neonates with borderline left ventricular hypoplasia. Echocardiography does not accurately measure left ventricular hypoplasia in these patients and may unfairly preclude some patients from a biventricular repair in whom magnetic resonance is reassuring.
Outcome and Growth Potential of Left Heart Structures After Neonatal Intervention for Aortic Valve Stenosis
2007, Journal of the American College of Cardiology
The purpose of this study was to determine trends of growth of left heart structures after intervention for neonatal aortic valve stenosis.
The growth potential of left heart structures in neonatal aortic valve stenosis after relief of obstruction might influence risk for subsequent outcomes.
From 1994 to 2004, 53 patients underwent neonatal (≤30 days old) balloon aortic valve dilation. Factors associated with time-related outcomes (death, reintervention, aortic valve replacement) and longitudinal changes in normalized left heart dimensions were sought.
The median age at intervention was 3.5 days (range 1 to 30 days). During a median follow-up of 3.2 years ranging up to 10.9 years, there were 31 reinterventions on the aortic valve in 21 (40%) patients and 7 deaths (13%). The presence of moderate or severe left ventricular (LV) endocardial fibroelastosis was the only independent predictor for time-related mortality (hazard ratio 22.1; p = 0.004), and a smaller initial aortic valve annulus z-score was a significant independent predictor for aortic valve replacement (hazard ratio 0.63 per 1-U change; p = 0.007). Aortic valve annulus, aortic sinus, and LV dimension z-scores significantly increased over time, whereas mitral valve z-scores remained below normal. The structure’s initial z-score and concomitant size of other left heart structures were significant independent factors associated with subsequent z-scores.
There is potential catch-up growth of the aortic valve and LV over time for neonates after intervention for aortic valve stenosis. However, the continued hypoplasia of the mitral valve warrants further consideration in the long-term management of these patients.
Aortic Atresia or Severe Left Ventricular Outflow Tract Obstruction with Ventricular Septal Defect: Results of Primary Biventricular Repair in Neonates
2006, Annals of Thoracic Surgery
Citation Excerpt :
Although the patients in the current report had very abnormal aortic valves, the mitral valve and LV dimensions fell within the normal range (−2 <z scores < 2). Even in the setting of modest LV hypoplasia, there appears to be the potential for LV growth [5–7, 13]. McElhinney and colleagues [5] in their review of 113 neonates who had undergone balloon valvuloplasty were able to demonstrate that LV end-diastolic dimensions normalized within the first 1 to 2 years of life.
Aortic atresia or severe aortic stenosis and left ventricular outflow tract obstruction is a frequent component of complex congenital heart disease. Aortic atresia or severe aortic stenosis and left ventricular outflow tract obstruction with two adequate ventricles is sometimes treated by Norwood palliation followed by late biventricular repair. We reviewed our experience with primary biventricular repair in this group of neonates.
Retrospective review identified 17 neonates (10 males) with aortic atresia or severe left ventricular outflow tract obstruction with ventricular septal defect and an adequate left ventricle undergoing primary biventricular repair between 1986 and 2002. Mean age was 7.7 ± 2.9 days, weight 3.3 ± 0.7 kg, and body surface area 0.21 ± 0.04 kg/m². Associated anomalies included arch hypoplasia, 7 (41%); aortic atresia, 7 (41%); and coarctation, 5 (29%). Results are reported as mean ± standard deviation.
Median follow-up was 6 years (range, 1 to 17.7 years). Three of the 17 (18%) died within 30 days. There were no deaths in this series since 1992. Nine patients (38.9%) required one reoperation, 7 of which were for conduit stenosis, 1 for left ventricular outflow tract obstruction, and 1 for residual ventricular septal defect with left ventricle–to–right atrium shunt. Freedom from death at 10 years was 82% by Kaplan–Meier estimate.
Excellent long-term survival can be achieved by primary biventricular repair as corroborated by our survival rate of 82%. Primary biventricular repair is an effective operation for aortic atresia and severe left ventricular outflow tract obstruction with adequate sized left ventricle that avoids interstage attrition associated with Norwood palliation and is our procedure of choice.

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Original ArticlesLeft ventricular growth in selected hypoplastic left ventricles: outcome after repair of coarctation of aorta

Abstract

Section snippets

Patients and methods

Results

Comment

Am J Cardiol

J Thorac Cardiovasc Surg

Ann Thorac Surg

J Thorac Cardiovasc Surg

J Thorac Cardiovasc Surg

J Am Coll Cardiol

J Thorac Cardiovasc Surg

Ann Thorac Surg

J Am Coll Cardiol

Am Heart J

Original Articles
Left ventricular growth in selected hypoplastic left ventricles: outcome after repair of coarctation of aorta