Br Heart3' 1994;71:370-371

Transcatheter coil embolisation of a pulmonaryarteriovenous malformation in a neonate

R Mark Grady, Angela M Sharkey, Nancy D Bridges

AbstractPulmonary arteriovenous malformations(PAVM) are a rare cause of cyanosis inneonates. A large PAVM in a neonate wassuccessfully occluded by transcatheterembolisation. At six months follow up thePAVM was undetectable and no new

lesions were found. Transcatheterembolisation should be considered as theprimary treatment for a PAVM in a childofany age.

(Br Heart3' 1994;71:370-371)

Division of PediatricCardiology,Department ofPediatrics,WashingtonUniversity School ofMedicine, St Louis,Missouri, USAR M GradyAM SharkeyN D BridgesCorrespondence to:Dr Ronald Mark Grady,Division of PediatricCardiology, St LouisChildren's Hospital, OneChildren's Place. St Louis,Missouri 63110, USA.

Figure 1 Angiogram showing balloon occlusion of theright pulmonary artery in a patient with a largepulmonary arteriovenous malformation. (B) Venous phaseshowing drainage of the pulmonary arteriovenousmalformation into the right lower pulmonary vein.

Pulmonary arteriovenous malformations(PAVM) are a rare cause of cyanosis inneonates. In all previously reported casessurgery was offered as the primary treatment.'We report the successful primary treatment ofa PAVM in a newborn by transcatheter coilembolisation.

Case reportAfter an uncomplicated labour and delivery, afull term, 2-7 kg infant was described in thedelivery room as "ashen grey". Examinationshowed no evidence of respiratory distress orcongestive heart failure. When placed in100% oxygen, the transcutaneous oxygen sat-urations were 80%-85% and the Po, was42 mm Hg. Because of increasing cyanosisthe child was intubated and transferred to ourinstitution on the third day after birth.

Cardiac examination found normal heartsounds with no murmur. Lung fields wereclear with no bruits. There was nohepatomegaly. Pulses and perfusion werenormal. No telangiectatic lesions were noted.The child's cyanosis did not improve withmechanical ventilation and neuromuscularblockade.A chest x ray film showed cardiomegaly,

oligaemic lung fields, and a right lower lobeopacity. Echocardiography showed bidirec-tional flow at the foramen ovale. Both ventri-cles were dilated. On both the subcostal andhigh parasternal views an echodense masswith multiple areas of vascular flow was seenposterior and inferior to the right pulmonaryartery in the hilum of the right lung. Dopplerechocardiography in these small vesselsshowed continuous flow. The right lower pul-monary vein was dilated. A diagnosis of aPAVM was made.On the fifth day after birth the child under-

went cardiac catheterisation. The right heartpressures were normal, with a right pul-monary artery pressure of 33/12 mm Hg,mean 21 mm Hg. During intubation and in100% oxygen, the arterial Po2 was 27 mm Hgwith a saturation of 77%. When the right pul-monary artery was occluded by a balloon thearterial Po, rose to 256 mm Hg. Anangiogram of the right pulmonary showed alarge, single PAVM with two afferent vesselsfrom the right lower lobe pulmonary artery,and one large efferent vessel draining to theright lower pulmonary vein (fig 1). The rightpulmonary artery was otherwise normal. EightGianturco steel coils were placed in the affer-ent vessels of the PAVM. Repeat angiography

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Transcatheter coil embolisation ofa pulmonary arteriovenous malformation in a neonate

pulmonary artery showing occlusion of the pulmonary)pen arrows) with coils and also coils in the bronchial

showed nearly complete occlusion. Therewere no complications. Within 36 hours ofthe procedure the child was extubated toroom air with transcutaneous oxygen satura-tions of 93%-100%.

At follow up six months later the child hadnormal growth and development with trans-cutaneous oxygen saturations of > 95% inroom air. Catheterisation showed normalhaemodynamic function. In room air the arte-rial Po2 was 96 mm Hg with a saturation of96%. Angiography showed no flow in thePAVM (fig 2) and normal right and left pul-monary arteries. No other PAVM were seen

in either lung. Two 2 mm bronchial collater-als supplying the right lower lobe wereembolised by coils.

DiscussionCongenital PAVMs are rarely diagnosed inneonates. Patients with PAVM typically

present in the second decade of life withcyanosis and dyspnoea. Of the 11 reportedneonatal cases, including this one, 10 pre-sented with cyanosis within the first 72 hoursafter birth.' The other infant presented atthree weeks of age with dyspnoea from ahemothorax after rupture of the PAVM.3Up to half of the adult patients with

PAVMs have multiple lesions, especially thosewith hereditary haemorrhagic telangiectasia(Osler-Weber-Rendu syndrome).2 All 11 ofthe neonates with PAVM had a single lesion,usually in the right or left lower lobe. Thedevelopment ofnew lesions has been reportedto occur in older children4; however, none ofthe previous surviving neonatal cases havereported follow up. Our patient had no newPAVM at six months. This uncertain naturalhistory emphasises the need for subsequentevaluation in all infants diagnosed withPAVM.

In the 10 previously reported neonatalcases, surgical resection was offered as the pri-mary treatment and in most cases (seven of10) resulted in a complete lobectomy.Transcatheter embolisation of PAVM hasproved to be safe and effective in older chil-dren and adults2 5; our case shows that thistechnique can be successfully used inneonates as well. Benefits include avoiding athoractomy, preserving normal lung tissuethat otherwise might be excised, the potentialto identify and occlude multiple, bilateralPAVMs in one procedure, and the ability toeasily repeat the procedure should new lesionsdevelop. Transcatheter embolisation shouldbe considered as the primary treatment ofPAVM in a child of any age.

1 Allen SW, Whitfield JM, Clarke DR, Sujansky E, WigginsJW. Pulmonary arteriovenous malformation in the new-born: a familial case. Pediatr Cardiol 1993;14:58-61.

2 Burke CM, Safai C, Nelson DP, Raffin TA. Pulmonaryarteriovenous malformations: a critical update. Am RevRespir Dis 1986;134:334-9.

3 Milovic I, Oluic D. Congenital pulmonary arteriovenousfistula with haemothorax in a neonate. Zeitschrift furKinderchinurgie 1989;44: 109-10.

4 Knight WB, Bush A, Busst CM, Haworth SG, Bowyer JJ,Shinebourne EA. Multiple pulmonary arteriovenousfistulas in childhood. IntJ7 Cardiol 1989;23:105-16.

5 Marin-Garcia J, Lock JE. Catheter embolization of pul-monary arteriovenous fistulas in an infant. PediatrCardiol 1992;13:41-3.

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