Fetal and Neonatal Presentation of Noncompacted Ventricular Myocardium: Expanding the Clinical Spectrum

Menon, Shaji C.; O’Leary, Patrick; Wright, Gregory B.; Rios, Rodrigo; MacLellan-Tobert, Susan G.; Cabalka, Allison K.

doi:10.1016/j.echo.2007.04.028

Cited by 33 publications

(46 citation statements)

References 18 publications

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“…Menon and colleagues noted that the noncompacted layer becomes more prominent with improvement of cardiac function and cardiomegaly during the clinical course of fetal-onset LVNC. 27 It is worth noting that 73% of infantile cases have cardiomegaly, and the N/C ratio might become lower in those cases with DCM hemodynamics, as a consequence of dilatation of the LV and thinning of the LV wall. In contrast to adult patients, 28 the N/C ratio of a mean of 5 segments had no correlation with LVEF.…”

Section: Discussionmentioning

confidence: 99%

Long-Term Prognosis of Patients With Left Ventricular Noncompaction　― Comparison Between Infantile and Juvenile Types ―

Wang

Takasaki

Ozawa

et al. 2017

Circ J

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Background:The natural history of left ventricular noncompaction (LVNC) is largely unsolved, so the aim of the present study was to clarify the clinical features and long-term prognosis of children with LVNC until adulthood. Methods and Results:We conducted a nationwide survey over 20 years and compared the clinical features, anatomical characteristics and long-term prognosis of 205 patients divided into 2 classifications: infantile type (diagnosed at <1 year of age: 108 cases) and juvenile type (diagnosed 1-15 years of age: 97 cases). Most patients diagnosed during infancy had heart failure (HF) at initial presentation (60.19%), while the majority of juvenile cases were asymptomatic (53.61%) but their event-free survival rate decreased gradually, because of later HF, thromboembolism and fatal arrhythmias. The initial LVEF was significantly lower in the infantile type and correlated with the thickness of the compacted layer in the LV posterior wall (LVPWC) and LV end-diastolic dimension (LVDD) Z-score, but not to the noncompacted to compacted layer (N/C) ratio. Survival analysis showed prognosis was similarly poor for both types after 2 decades. The significant risk factors for death, heart transplantation or implantable cardioverter-defibrillator insertion were congestive HF at diagnosis and lower LVPWC Z-score but not age of onset.Conclusions: LVNC of both types showed poor long-term prognosis, therefore ongoing follow-up is recommended into adulthood. HF at diagnosis and LVPWC hypoplasia are major determinants of poor prognosis.

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Section: Discussionmentioning

confidence: 99%

Long-Term Prognosis of Patients With Left Ventricular Noncompaction　― Comparison Between Infantile and Juvenile Types ―

Wang

Takasaki

Ozawa

et al. 2017

Circ J

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“…In 85 cases, LVHT was diagnosed by fetal echocardiography and postnatally confirmed by either echocardiography or autopsy. In the remaining three cases, LVHT was diagnosed only postnatally, but review of the fetal echocardiograms disclosed that LVHT was visible but had been overlooked [35,38,43].…”

Section: Cases With Prenatal Diagnosis Of Lvhtmentioning

confidence: 95%

“…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%

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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“…However, there is an increasing number of reports of prenatal diagnosis of LVNC using fetal echocardiography. 23,24 These findings call into question the theory that arrested embryonic development underlies the pathogenesis of LVNC.…”

Section: Etiology and Pathogenesismentioning

confidence: 99%

Left Ventricular Noncompaction

Ichida

2009

Circ J

114

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Left ventricular noncompaction (LVNC) is a recently defined cardiomyopathy characterized by a pattern of prominent trabecular meshwork and deep intertrabecular recesses, and is thought to be caused by arrest of normal endomyocardial morphogenesis. Although LVNC has been classified as a primary cardiomyopathy of genetic origin, its definition and diagnostic criteria are still being debated. Isolated LVNC was thought to be rare; however, heightened awareness has resulted in an increased detection of the morphological features of LVNC in routine clinical practice, especially in the adult population. Clinical manifestations are highly variable, ranging from no symptoms to disabling congestive heart failure, arrhythmias, and systemic thromboemboli. LVNC, like other forms of inherited cardiomyopathy, is genetically heterogeneous and can be inherited as an autosomaldominant or X-linked recessive disorder. It has been linked to mutations in several genes, including LIM domain binding protein 3 (ZASP), α-dystrobrevin (DTNA), tafazzin (TAZ/G4.5) and those encoding sarcomeric proteins. However, the relatively small contribution of known mutations to the disease, compared with the higher proportion of familial cases suggests that other elusive genes remain to be identified. (Circ J 2009; 73: 19 -26)

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Fetal and Neonatal Presentation of Noncompacted Ventricular Myocardium: Expanding the Clinical Spectrum

Cited by 33 publications

References 18 publications

Long-Term Prognosis of Patients With Left Ventricular Noncompaction　― Comparison Between Infantile and Juvenile Types ―

Long-Term Prognosis of Patients With Left Ventricular Noncompaction　― Comparison Between Infantile and Juvenile Types ―

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

Left Ventricular Noncompaction

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Fetal and Neonatal Presentation of Noncompacted Ventricular Myocardium: Expanding the Clinical Spectrum

Cited by 33 publications

References 18 publications

Long-Term Prognosis of Patients With Left Ventricular Noncompaction ― Comparison Between Infantile and Juvenile Types ―

Long-Term Prognosis of Patients With Left Ventricular Noncompaction ― Comparison Between Infantile and Juvenile Types ―

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

Left Ventricular Noncompaction

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Product

Resources

About

Long-Term Prognosis of Patients With Left Ventricular Noncompaction　― Comparison Between Infantile and Juvenile Types ―

Long-Term Prognosis of Patients With Left Ventricular Noncompaction　― Comparison Between Infantile and Juvenile Types ―