Prenatal Diagnosis of Isolated Ventricular Noncompaction of the Myocardium

Sleurs, E.; Catte, Luc De; Benatar, Abraham

doi:10.7863/jum.2005.24.9.1325

Cited by 14 publications

(19 citation statements)

References 25 publications

(24 reference statements)

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“…In 32 cases (36 %), no additional cardiac abnormalities were reported [1,3,28,30,32,35,38,39,41,43,44,48,49,52,57,60,62]. In three further cases, LVHT was associated with a left atrial thrombus [43], myocardial calcification [45] and restrictive cardiomyopathy [24].…”

Section: Cases With Prenatal Diagnosis Of Lvhtmentioning

confidence: 90%

“…Before the year 2000, only one further report was published [62]. Between 2000 to 2009, 29 cases (33 %) were published [1,7,12,17,24,32,38,39,43,44,48,49]. Since 2010, 57 cases (65 %) were published [3,4,14,28,30,41,45,47,52,55,57,58,60].…”

Section: Cases With Prenatal Diagnosis Of Lvhtmentioning

confidence: 96%

“…In two cases, VHT was confined to the right ventricle exclusively [1,52]. In 29 further cases (33 %), both ventricles were affected [3,7,12,14,24,28,32,38,39,41,43,48,49,52,55,57,58,60,62]. In the remaining 57 cases (65 %), VHT was confined to the left ventricle.…”

Section: Cases With Prenatal Diagnosis Of Lvhtmentioning

confidence: 97%

“…The majority of the cases (n = 49, 56 %) were reported from hospitals in the USA [1,3,4,7,12,17,24,30,35,38,57,58]. Nine cases were reported from China [52], eight cases from Canada [60], five cases from France [39,62], five cases from Turkey [43], three cases form the UK [32,48], two cases each from Italy and the Netherlands [28,44,45] and one case each from Ireland, Greece, Portugal, Japan and Belgium [14,41,47,49,55]. The ethnicity is only reported in three cases of African origin [32,39,47].…”

Section: Cases With Prenatal Diagnosis Of Lvhtmentioning

confidence: 98%

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Fetal Ventricular Hypertrabeculation/Noncompaction: Clinical Presentation, Genetics, Associated Cardiac and Extracardiac Abnormalities and Outcome

2015

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Left ventricular hypertrabeculation/noncompaction (LVHT) is a cardiac abnormality of unknown etiology. Aim of the review was to summarize the current knowledge about fetal LVHT, including clinical presentation, associated cardiac and extracardiac abnormalities and outcome. In 88 cases, LVHT was diagnosed by fetal echocardiography. In 36 %, no additional cardiac abnormalities were reported; in the remaining 64 %, one or more cardiac abnormalities were reported. Eight cases died prenatally, 17 were electively terminated, and 24 patients died after birth. Six patients were lost to follow-up, and 33 patients are alive at a mean age of 26 months. Surviving cases presented less frequently with fetal hydrops (13 vs. 62 %, p = 0.0004), complete heart block (27 vs. 78 %, p = 0.0076), more than three associated cardiac abnormalities (9 vs. 47 %, p = 0.0008) and more frequently with isolated LVHT (52 vs. 19 %, p = 0.009) than cases who died. Of the surviving patients, 16 received pharmacotherapy, three received pacemakers, eight underwent surgical procedures and four underwent heart transplantation. Postnatal regression of left ventricular hypertrophy and development of LVHT was found in four cases, improvement in cardiac function in two, and regression of right VHT in two. At autopsy, endocardial fibrosis was the most frequent abnormality in 92 %. Thirty-eight percentage of cases with fetal LVHT survived. Fetal and postnatal echocardiographic findings challenge the "embryonic pathogenetic" hypothesis of LVHT. Furthermore, fetal pathoanatomic findings like endocardial fibrosis might play a role in clarifying the still unsolved pathogenesis of LVHT.

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Section: Cases With Prenatal Diagnosis Of Lvhtmentioning

confidence: 90%

Section: Cases With Prenatal Diagnosis Of Lvhtmentioning

confidence: 96%

Section: Cases With Prenatal Diagnosis Of Lvhtmentioning

confidence: 97%

Section: Cases With Prenatal Diagnosis Of Lvhtmentioning

confidence: 98%

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Fetal Ventricular Hypertrabeculation/Noncompaction: Clinical Presentation, Genetics, Associated Cardiac and Extracardiac Abnormalities and Outcome

2015

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“…Prenatal diagnosis of VNC can usually be made by a standard fetal anatomic sonographic 4-chamber view or by fetal echocardiography; the earliest prenatal sonographic 17 A review of the literature revealed 5 previously published reports comprising a total of 15 cases in which the diagnosis of VNC was made prenatally (Table 1). [17][18][19][20][21] More than half of the ongoing pregnancies (5 were terminated) resulted in fetal or neonatal death. Only 4 of 10 ongoing pregnancies had live births.…”

Section: -14mentioning

confidence: 99%

Prenatal Diagnosis of Omphalocele and Left Atrial Isomerism (Polysplenia) Including Complex Congenital Heart Disease With Ventricular Noncompaction Cardiomyopathy

Boe

Rhee-Morris

Towner

et al. 2008

Journal of Ultrasound in Medicine

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e report prenatal diagnosis of a rare constellation of findings, including omphalocele and polysplenia (left atrial isomerism [LAI]) with cardiac malformations including ventricular noncompaction (VNC) cardiomyopathy. The heterotaxy syndromes (polysplenia or LAI and asplenia or right atrial isomerism) are rare syndromes in which organs that are usually asymmetric are abnormally symmetric or abnormally positioned. Complex congenital heart disease is frequently associated with heterotaxy, with the heart being substantially affected in both structure and orientation. Heterotaxy has also been occasionally associated with a rare type of cardiomyopathy: VNC, described by Feldt et al 1 and Ozkutlu et al. 2 Omphalocele is a relatively common birth defect that is due to failure of the abdominal wall to close in association with return of the bowel in the first trimester. We report a case in which all of these findings were present. The cardiac findings were previously included in a pathology series on LAI with VNC by Friedberg et al 3 ; however, to our knowledge, prenatal diagnosis of this unique collection of findings has not been reported previously. Case ReportA 21-year-old multiparous woman was referred at 21 weeks' gestation for evaluation of fetal bradycardia, which had been first noted at an office visit 9 weeks earlier. The fetal heart rate was noted to be 90 to 110 beats per minute at 12 weeks and remained in this range at reevaluations at 14 and 18 weeks. Her expanded maternal serum α-fetoprotein (AFP) triple-marker screening test returned an elevated AFP value of 3.01 multiples of the median.

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Left Ventricular Noncompaction

Sarma¹

2019

Cardiac Problems in Pregnancy, 4th Edition

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Prenatal Diagnosis of Isolated Ventricular Noncompaction of the Myocardium

Cited by 14 publications

References 25 publications

Fetal Ventricular Hypertrabeculation/Noncompaction: Clinical Presentation, Genetics, Associated Cardiac and Extracardiac Abnormalities and Outcome

Fetal Ventricular Hypertrabeculation/Noncompaction: Clinical Presentation, Genetics, Associated Cardiac and Extracardiac Abnormalities and Outcome

Prenatal Diagnosis of Omphalocele and Left Atrial Isomerism (Polysplenia) Including Complex Congenital Heart Disease With Ventricular Noncompaction Cardiomyopathy

Left Ventricular Noncompaction

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