Echocardiographic Diagnosis and Prognosis of Fetal Left Ventricular Noncompaction




Background


Left ventricular noncompaction (LVNC) has rarely been described in the fetus.


Methods


The presence of associated congenital heart disease and rhythm disturbance was identified and the presence of heart failure was assessed using the cardiovascular profile score in all fetuses with LVNC presenting from January 1999 to July 2010. The left ventricle was divided into 12 segments—four segments each at the base, midpapillary, and apical regions—in the short-axis view to calculate the noncompaction/compaction ratio for each segment.


Results


Of 24 fetuses with LVNC included in the study, 22 had significant congenital heart disease, and 15 had complete heart block. Of the 16 patients with adequate follow-up and not electively terminated, 12 (81%) died or progressed to heart transplantation. The average noncompaction/compaction ratios were 2.02 in patients who died or underwent heart transplantation and 1.67 in survivors ( P = .2034). Fifty-seven of 93 measured segments (61%) of the left ventricle in the patients who died or underwent heart transplantation had noncompaction/compaction ratios ≥ 2 compared with five of 17 measured segments (29%) in survivors ( P = .0837). The average cardiovascular profile score was 6. The apical region had greater involvement of noncompaction than the midpapillary and basal regions, with ratios of 2.27, 2.14, and 1.10, respectively ( P = .00035).


Conclusions


Fetuses with LVNC have a poor prognosis that may be related to associated congenital heart disease, increased segmental involvement of noncompaction, and complete heart block and can be predicted by the cardiovascular profile score.


Left ventricular noncompaction (LVNC) is a disease that may represent a failure of normal embryonic myocardial maturation, resulting in the persistence of trabeculated, loosely interwoven fibers or “spongy” myocardium. Despite the ability to recognize LVNC in the fetus, it is rarely described in this population. Many studies have discussed the use of echocardiography to explain the lesion in adults and children, identifying prominent trabeculations and the utility of the noncompaction/compaction ratio in diagnosing this lesion. The noncompaction/compaction ratio is determined by measuring the depth of noncompaction and compaction zones in systole from a short-axis or apical view ( Figure 1 ). However, although distinct criteria have been established for the diagnosis of LVNC after birth, criteria for diagnosis in the fetus have not been established.




Figure 1


Diagram of 12-segment analysis of the left ventricle from the short-axis (SAX) view and example of noncompaction (NC) and compaction (C) zones at the level of the apex (AP). The three diagrams at the bottom illustrate the modified version of the American Heart Association and American Society of Echocardiography’s 16 segments of the left ventricle from the short-axis view at the level of the mitral valve (MV), papillary muscles (PM), and apex. An example of the noncompaction and compaction zones of the left ventricle is demonstrated at the level of the apex from our institution (patient 1). The noncompaction/compaction ratio was measured at end-systole for each of the 12 segments for all patients included in the study. Left ventricle segmental diagrams courtesy of Cerqueira et al. have been modified to conform to the described limitations of fetal imaging. ANT , Anterior; INF , inferior; LAT , lateral; RV , right ventricle; SEPT , septal.


The objectives of this study were to analyze echocardiographic data to (1) establish the criteria for diagnosis of fetal LVNC on the basis of a 12-segment modification of the standards adopted by the American Heart Association and American Society of Echocardiography, (2) identify associated anatomic and physiologic consequences, (3) determine the severity of noncompaction using the noncompaction/compaction ratio, and (4) define outcomes to assess prognosis.


Methods


Demographic Data Collection


We retrospectively reviewed our experience with fetuses diagnosed with LVNC at Lucile Packard Children’s Hospital at Stanford, California, and The Heart Institute for Children at Hope Children’s Hospital in Oak Lawn, Illinois, from January 1999 to July 2010 using the Siemens syngo Dynamics workstation (Siemens Medical Solutions USA, Inc., Ann Arbor, MI). Demographic data included gestational age at presentation and gender. The collected outcome data included physiologic consequences, rhythm disturbances, hydrops fetalis, death, and termination. Available postnatal echocardiograms and necroscopy studies were reviewed to confirm the diagnosis of noncompaction ( Table 1 ). This study was approved through our institutional review board (Protocol 17803), and all data were made anonymous in accordance with the Health Insurance Portability and Accountability Act.



Table 1

Associated pathology, rhythm, clinical outcome of LVNC diagnosed in utero























































































































































































































































































Patient Diagnosis Cardiac position and situs Gender Gestational age (wk) Pathology Rhythm Hydrops Outcome Postnatal studies
1 LVNC, RVNC Dextrocardia, situs solitus Female 28 DORV, TR, PS, AS, interrupted IVC CHB Yes Died Autopsy
2 LVNC Levocardia, situs solitus Female 26 + 2 Moderate MR, mild RVH Sinus bradycardia No Died Echocardiography
3 LVNC, RVNC Levocardia, situs solitus Male 29 + 1 AVSD, DORV, PS, AS, interrupted IVC, RVH CHB Yes Died Echocardiography
4 LVNC Levocardia, situs solitus Unknown 17 DORV with d-MGA, pulmonary atresia CHB No Terminated None
5 LVNC Levocardia, situs solitus Female 15 AVSD, severe MR, pulmonary atresia CHB Yes Terminated None
6 LVNC Levocardia, situs solitus Unknown 18 DORV, mitral atresia, aortic arch interruption type B Sinus No Lost to follow-up None
7 LVNC Dextrocardia, situs solitus Female 29 + 2 AVSD, DORV, RV hypoplasia, AS, interrupted IVC Sinus No Lost to follow-up None
8 LVNC Left atrial isomerism Male 30 AVSD, pulmonary atresia, RV hypoplasia, interrupted IVC Sinus bradycardia No Died Echocardiography
9 LVNC Levocardia, situs solitus Female 21 + 4 None Sinus Yes Alive, metastatic osteosarcoma Echocardiography
10 LVNC Dextrocardia, situs solitus Female 33 AVSD, DORV, RVH, interrupted IVC, coarctation of the aorta CHB Yes Lost to follow-up None
11 LVNC Dextrocardia, situs solitus Female 32 DORV with d-MGA, crisscross AV valves, severe TR, mild MR Sinus No Alive, underwent heart transplantation Echocardiography
12 LVNC Levocardia, situs solitus Male 18 + 1 AVSD, DORV with d-MGA, AS, RV hypoplasia, RVH, hypoplastic aortic valve, hypoplastic aortic arch CHB No Alive Echocardiography
13 RVNC, LVNC Levocardia, situs solitus Male 22 AVSD, DORV with d-MGA CHB Yes Died Autopsy
14 LVNC Dextrocardia, situs solitus Unknown 17 AVSD High second-degree heart block No Lost to follow-up None
15 LVNC Levocardia, situs solitus Male 34 AVSD, DORV with l-MGA, interrupted IVC, LSVC coronary sinus, PS, AS CHB Yes Died None
16 LVNC Left atrial isomerism Female 28 AVSD, DORV with d-MGA, PS Second-degree heart block Yes Died Autopsy
17 LVNC Left atrial isomerism Male 37 AVSD CHB No Died None
18 LVNC Levocardia, situs solitus Female 36 AVSD, DORV CHB No Alive None
19 LVNC Dextrocardia, left atrial isomerism Unknown 22 + 3 AVSD, interrupted IVC CHB Yes Lost to follow-up None
20 LNVC Dextrocardia, left atrial isomerism Female 29 AVSD, PS CHB No Died Autopsy
21 LVNC Dextrocardia, left atrial isomerism Female 35 AVSD, DORV, interrupted IVC, PS CHB No Died None
22 LVNC Dextrocardia, situs solitus Unknown 21 AVSD Sinus No Lost to follow-up None
23 LVNC Left atrial isomerism Female 18 DORV CHB No Died Autopsy
24 LVNC Levocardia, situs solitus Female 38 AVSD, DORV CHB No Died None

AS , Aortic stenosis; AVSD , atrioventricular septal defect; CHB , complete heart block; DORV , double-outlet right ventricle; IVC , inferior vena cava; LSVC , left superior vena cava; MGA , malposed great arteries; MR , mitral regurgitation; PS , pulmonary stenosis; RVH , right ventricular hypertrophy; RVNC , right ventricular noncompaction; TR , tricuspid regurgitation.


Echocardiographic Data Collection


Ultrasound equipment included the Siemens Acuson C512 revision 12.0 (Siemens Medical Solutions USA, Inc., Mountain View, CA) and the Phillips iE33 (Philips Medical Systems, Bothell, WA). All fetal echocardiographic records were reviewed by two reviewers (A.A. and R.P.). Strict criteria were used to ensure the appropriate diagnosis; all fetuses had the following left ventricular findings as described by Jenni et al. : (1) multiple trabeculations and recesses, (2) distinct compacted and noncompacted layers, and (3) a noncompaction/compaction ratio ≥ 2.0 during systole. Given the retrospective nature of this study, we first identified patients with a diagnosis of LVNC in the report and then reviewed the echocardiograms to ensure that the patients met all inclusion criteria. The first complete fetal echocardiogram for the patient was included in the study. Studies with incomplete imaging and poor quality were excluded from analysis.


To determine the severity of noncompaction, we calculated the noncompaction/compaction ratio in the short-axis view of the left ventricle for four segments each at the level of the mitral valve, papillary muscle, and apex, which was an abbreviated version of the 16-segment model described by the American Society of Echocardiography and the American Heart Association as a result of the limitations in resolution on fetal echocardiography ( Figure 1 ). We subsequently calculated the average noncompaction/compaction ratio for each segment ( Table 2 ). Segments that were missing because of ventricular septal defects or that were not well visualized were not included in the average. Segments were given a score of zero when there was no evidence of noncompaction. One reviewer (R.P.) analyzed all segments blinded to fetal outcomes to reduce bias in measurements.



Table 2

Noncompaction/compaction ratios in basal, midpapillary, and apical segments of the left ventricle on fetal echocardiography


















































































































































































































































































































































































































































































Patient Base Midpapillary Apex
AS AL IS IL Average AS AL IS IL Average AS AL IS IL Average
1 VSD 2.14 VSD 2.27 2.20 2.27 0.00 1.00 3.03 1.58 1.21 2.79 1.71 3.77 2.37
2 0.00 0.00 0.00 0.00 0.00 0.00 2.72 0.00 0.55 0.82 NI NI NI NI NA
3 0.00 0.00 0.00 0.00 0.00 VSD 2.43 VSD 0.03 1.23 1.00 1.61 1.96 2.41 1.74
4 VSD 2.00 VSD 3.30 2.65 1.00 1.81 2.27 1.50 1.65 NI NI NI NI NA
5 VSD 0.00 VSD 0.00 0.00 VSD 1.54 VSD 2.50 2.02 VSD 2.11 2.09 1.83 2.01
6 VSD 0.00 VSD 0.00 0.00 VSD 2.05 1.52 2.12 1.90 2.00 1.64 2.04 2.17 1.96
7 VSD 2.68 1.26 2.59 2.18 VSD 2.56 1.00 2.79 2.12 2.43 3.21 2.63 3.67 2.98
8 VSD VSD 1.12 2.67 1.89 0.30 2.20 2.79 2.25 1.88 0.85 1.33 NI 3.04 1.74
9 0.00 0.00 0.00 0.00 0.00 0.64 1.00 1.50 1.56 1.18 0.68 1.94 1.47 1.64 1.43
10 NI NI NI NI NA NI NI NI NI NA NI NI NI NI 2.04 ∗
11 † NI NI NI NI NA NI NI NI NI NA NI NI NI NI NA
12 † NI NI NI NI NA NI NI NI NI NA NI NI NI NI NA
13 NI NI NI NI NA 2.00 3.17 VSD 2.33 2.50 1.33 2.33 1.67 2.33 1.92
14 NI NI NI NI NA 3.00 3.00 2.25 2.00 2.56 5.33 2.25 2.80 3.60 3.50
15 1.67 3.00 2.33 3.25 2.56 3.33 4.75 5.33 2.18 3.90 3.00 3.50 2.18 3.50 3.05
16 NI NI NI NI NA 4.00 4.00 2.53 3.67 3.55 2.80 2.29 1.67 2.75 2.38
17 NI NI NI NI NA VSD 2.29 2.50 2.80 2.53 NI NI NI NI 3.20 ∗
18 NI NI NI NI NA 3.75 4.40 2.40 2.17 3.18 NI NI NI NI 4.25 ∗
19 NI NI NI NI NA NI NI NI NI NA NI NI NI NI 2.25 ∗
20 † NI NI NI NI NA NI NI NI NI NA NI NI NI NI NA
21 0.83 0.00 VSD 1.00 0.61 3.14 1.88 2.00 1.29 2.08 2.58 2.00 3.00 2.50 2.52
22 0.83 0.70 NI 1.65 1.06 1.35 1.75 NI 1.88 1.66 2.33 1.31 2.76 2.67 2.27
23 NI NI NI NI NA 2.00 2.32 2.25 2.19 2.19 1.95 1.53 1.81 2.21 1.88
24 NI NI NI NI NA VSD 2.00 3.00 2.44 2.48 2.00 3.11 1.80 3.11 2.51
Segmental average 0.55 0.96 0.78 1.39 2.06 2.44 2.10 2.05 2.12 2.13 2.14 2.72
Base total average 1.10 Midpapillary total average 2.14 Apex total average 2.27
Base SD 1.12 Midpapillary SD 0.81 Apex SD 0.58

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Jun 11, 2018 | Posted by in CARDIOLOGY | Comments Off on Echocardiographic Diagnosis and Prognosis of Fetal Left Ventricular Noncompaction

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