The role of echocardiography in prenatal diagnosis of pulmonary arteriovenous malformation

Sinkovskaya, Elena; Berkley, Eliza; Bogdan, Donna; Sclater, A.; Abuhamad, Alfred

doi:10.1002/pd.2261

Cited by 10 publications

(14 citation statements)

References 9 publications

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“…Prenatal diagnosis of PAVM has been described previously3–6. Heling and colleagues3 described a case of intrapulmonary arteriovenous malformation diagnosed at 27 weeks' gestation.…”

Section: Discussionmentioning

confidence: 99%

Prenatal diagnosis of a pulmonary arteriovenous malformation

Tamir¹,

Malinger²

2012

Ultrasound in Obstet & Gyne

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Pulmonary arteriovenous malformations (PAVMs) CASE REPORTPulmonary arteriovenous malformations (PAVMs) are caused by abnormal communications between pulmonary arteries and pulmonary veins, and are most commonly congenital in nature 1 . They may occur as an isolated anomaly or in association with hereditary hemorrhagic telangiectasia (HHT) (Rendu-Osler-Weber syndrome). The right-to-left shunting induces chronic hypoxemia, cyanosis, clubbing, polycythemia, paradoxical embolism and infections 2 . We describe a case of prenatal diagnosis of PAVM in a 16-week-old fetus.The patient presented for early targeted organ screening at 15 weeks' gestational age. The left side of the fetal heart was enlarged with a shift towards the left thorax; the left atrium was particularly enlarged. The tricuspid valve appeared to be wide with mild regurgitation, and the supravalvular aorta and pulmonary artery were slightly widened.Fetal echocardiographic examination at 16 weeks showed the left heart to be enlarged as compared to the right, particularly the left atrium as compared to the right atrium. At least one muscular ventricular septal defect was present. During two-dimensional gray-scale ultrasound examination, a jet was observed in the posterior portion of the left atrium flowing from one of the right pulmonary veins into the left atrium ( Figure 1a). Longitudinal color Doppler showed antegrade flow in the main pulmonary artery that appeared to be joined to the jet, whose flow direction was posterior-anterior. Applying four-dimensional ultrasound (4DUS) scanning a spatiotemporal image correlation (STIC) volume dataset with color Doppler was acquired. In post-processing multiplanar reconstruction (MPR) we observed in the apical four-chamber view that the jet crossed the mitral valve. With the aid of the navigation dot, we observed in all three orthogonal planes (apical four-chamber view, longitudinal view and coronal C-plane) the area of the junction of the pulmonary artery and what appeared to be a pulmonary vein (Figure 1b and supplementary Videoclip S1). Pulsed wave color Doppler was applied to this area and showed a typical triphasic flow pattern in the shunt, which confirmed it as a pulmonary vein. Flow in the shunt reached maximum velocity of 60 cm/s (Figure 1c). In light of these findings a diagnosis of PAVM was made. Owing to the poor prognosis for this kind of PAVM the parents opted for termination of pregnancy, which was performed by dilation and evacuation (precluding postmortem evaluation). DISCUSSIONPAVMs are direct communications between the smaller pulmonary arteries and veins. These vascular fistulas allow blood to bypass the capillary system, and flow from arteries directly into veins. These lesions are usually congenital and are caused by failure of differentiation of the embryonic vascular plexus. The low-resistance venous system is exposed to systemic pressures leading to abnormal vascular formations 2 . Since the first description at an

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“…Prenatal diagnosis of PAVM has been described previously3–6. Heling and colleagues3 described a case of intrapulmonary arteriovenous malformation diagnosed at 27 weeks' gestation.…”

Section: Discussionmentioning

confidence: 99%

Prenatal diagnosis of a pulmonary arteriovenous malformation

Tamir¹,

Malinger²

2012

Ultrasound in Obstet & Gyne

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“…Though it is a rare finding, prenatal diagnosis of PAVM has been previously described in a few case reports [2,4,7,8,9,10]. Corresponding to our case, the described 6 fetuses had isolated PAVM and no family history for HHT.…”

Section: Discussionmentioning

confidence: 54%

Large Fetal Pulmonary Arteriovenous Malformation Detected at Midtrimester Scan with Subsequent High Cardiac Output Syndrome and Favorable Postnatal Outcome

Hellmund

Berg

Bryan³

et al. 2013

Fetal Diagn Ther

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Prenatal diagnosis of pulmonary arteriovenous malformations (PAVM), caused by abnormal communications between pulmonary arteries and pulmonary veins, is rarely described. We report a case of a PAVM between the right pulmonary artery and the left atrium, referred to our prenatal unit at 22 + 1 weeks of gestation, with severe cardiomegaly, dilation of the right pulmonary artery and a right pulmonary vein and retrograde flow in the ductus arteriosus. The fistula was located in the right lung and showed a broad, disturbed flow at color Doppler with high velocity and low pulsatility. The fetus was monitored weekly and cardiac function remained sufficient until 36 + 1 weeks of gestation, when increasing cardiomegaly prompted delivery by cesarean section. The newborn was transferred to the neonatal intensive care unit, intubated and the fistula was surgically removed. The boy could be discharged at the 43rd day of life and showed an uneventful course until the last follow-up at the age of 4 years, with no residual mental or physical handicaps and a normal cardiac function. Despite adverse outcomes described in previously reported cases of large PAVM complicated by severe cardiomegaly at midtrimester scan, our case had a good outcome.

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“…In eight previous cases of prenatally diagnosed PAVMs, four infants survived after successful treatment (Table 1) [13456789]. Among four survived infants, one was diagnosed as late as 37 weeks.…”

Section: Discussionmentioning

confidence: 99%

“…They are quite rare and only a few reports of prenatal diagnosis have been described [123456789]. The cases diagnosed prenatally were reported as an isolated anomaly or in association with simple cardiac defects.…”

Section: Introductionmentioning

confidence: 99%

A case of prenatally diagnosed extrapulmonary arteriovenous malformation associated with a complex heart defect

Jeong

Won

et al. 2016

Obstet Gynecol Sci

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Pulmonary arteriovenous malformations are rare vascular anomalies of the lung, only a few cases of which have been diagnosed prenatally. The diagnostic clue for prenatal diagnosis was cardiomegaly with a particularly enlarged left atrium. All previous cases of pulmonary arteriovenous malformations diagnosed prenatally have been reported as an isolated anomaly or in association with simple heart defects. We here describe the first case of a pulmonary arteriovenous malformation with a complex heart defect that was diagnosed prenatally at 21.0 weeks of gestation and confirmed by postmortem autopsy.

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The role of echocardiography in prenatal diagnosis of pulmonary arteriovenous malformation

Cited by 10 publications

References 9 publications

Prenatal diagnosis of a pulmonary arteriovenous malformation

Prenatal diagnosis of a pulmonary arteriovenous malformation

Large Fetal Pulmonary Arteriovenous Malformation Detected at Midtrimester Scan with Subsequent High Cardiac Output Syndrome and Favorable Postnatal Outcome

A case of prenatally diagnosed extrapulmonary arteriovenous malformation associated with a complex heart defect

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