Prenatal ultrasound diagnosis of fetal isolated right ventricular noncompaction with pulmonary artery sling: A rare case report

Abstract

Noncompaction of the ventricular myocardium (NVM) is a cardiomyopathy characterized by abnormally enlarged intraventricular trabeculae and intersecting deep recesses. It is believed that the failure of reticular trabeculae compaction during early embryonic development plays an important role in the disease. Cavernous cardiomyopathy often involves the left ventricle, while right ventricle and bilateral ventricles are rare. Although there have been several studies on NVM in children and adults, there are few studies on fe-tuses. NVM can occur independently or in combination with other complex cardiac malformations. 1,2 In our case, a rare pulmonary artery sling was also found. The clinical course and prognosis of PAS mainly depend on the degree of airway stenosis. It is generally believed that the high mortality rate of PAS is due to the compression of the tracheobronchial tree rather than PAS itself. Two-thirds of the patients develop symptoms associated with airway diseases in the first month of life. PAS easily escapes detection in its early stages. If untreated, the first-year mortality rate could be approximately 90%.Therefore, precise prenatal diagnosis could be beneficial to its clinical treatment and prognosis. 3,4 Fetal echocardiography is the preferred method for diagnosing fetal cardiomyopathy and other cardiac malformations due to its noninvasive, safe, and inexpensive characteristics. It can provide reliable imaging diagnosis for prenatal consultation of pregnant women. 5 Our report demonstrated that IRVNC associated with PAS can be diagnosed in fetal life by prenatal ultrasound. | C A S E REP ORTA 31-year-old pregnant woman (gravida 2, para 1) was referred to our maternal-fetal medical center at 30 + 5 weeks for suspected heart defects. Her nuchal translucency test and prenatal cell-free fetal DNA testing were both felt to be low-risk. She reported no family history. Fetal echocardiography showed the right atrium and ventricle were markedly dilated, a maximum right ventricular wall thickness of 7.5 mm, and multiple prominent trabeculations and deep intertrabecular recesses communicating with the right ventricular cavity (Figure 1). Both the LV and RV function were normal.The left pulmonary artery anomalously originates from a normally positioned right pulmonary artery and running posterior to the trachea, forming a PAS. Color Doppler Flow Imaging (CDFI) revealed that there was a right-to-left blood flow signal in the foramen ovale, AbstractNoncompaction of the ventricular myocardium (NVM), also known as spongy myocardium, is a rare type of cardiomyopathy that has a serious impact on fetuses, children, and adults. NVM mainly affects the left ventricle, as isolated right ventricular noncompaction (IRVNC) is rare. Pulmonary artery sling (PAS) is a rare condition in which the left pulmonary artery anomalously originates from a normal positioned right pulmonary artery, and only a few studies have reported PAS in fetuses. Fetal IRVNC complicated with PAS has not been reported yet. Here, we report a case of ...

Anomalous origin of the fetal pulmonary artery

“…Therefore, when PAS is suspected during prenatal ultrasonography, the coronal section of the trachea should be scanned for abnormalities in morphology, course, and stenotic degree of the trachea. Available literature documents that about 40%–50% of PAS cases are complicated with other cardiovascular malformations ( 1 ) and that PAS can be associated with right ventricular myocardial insufficiency and total anomalous pulmonary venous connection ( 22 , 23 ). In the current study, 61.5% of PAS cases were accompanied by other intracardiac malformations, such as PLSVC, TOF, and VSD.…”

Diagnóstico prenatal de no compactación del miocardio ventricular: presentación de un caso

“…En la bibliografía consultada (1)(2)(3)(4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(18)(19) se resume las posibles causas de esta entidad, entre las que destacan proteínas con mutaciones genéticas, probablemente responsables de la aparición Figura 2. Curvas de deFormaCión y su respeCtiva tasa de deForma-Ción (strain y strain rate) de ambos ventríCulos del Caso Con la mioCardiopatía no CompaCtada (CírCulos rojos a y b), Con evidentes diFerenCias si se las Compara Con Caso Control normal (C y d, CírCulos verdes) de la misma edad gestaCional (31 semanas).…”

“…Engberding y Bender llamaron al trastorno 'persistencia de sinusoides miocárdicos aislados'. Puede ocurrir como una entidad aislada o asociada con otras patologías del corazón (11) , y a menudo puede afectar ambos ventrículos.…”