Scimitar syndrome is a rare cardiopulmonary malformation. Presentation in infancy is usually associated with pulmonary hypertension and severe symptoms. We discuss treatment of such an infant. Two abnormal systemic vessels supplying the sequestered lower part of the right lung were embolized using catheter-inserted coils. The patent arterial duct was surgically ligated. These procedures resulted in a significant reduction of the shunt and the level of pulmonary hypertension, as well as in an impresssive improvement of symptom

Kallfelz, Hans Carlo

Luhmer, Ingrid

Pfammatter, Jean-Pierre

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Cardiol Young 1997; 7: 454-457© Greenwich Medical Media Ltd.ISSN 1047-9511Brief ReportInfantile scimitar syndrome with severe pulmonaryhypertension: successful treatment with coil embolizationof the systemic arterial supply to the sequestered lungJean-Pierre Pfammatter, Ingrid Luhmer, Hans Carlo KallfelzDivision ofPediatric Cardiology, Children "s Hospital, Hannover Medical School Hannover, GermanyAbstract Scimitar syndrome is a rare cardiopulmonary malformation. Presentation in infancy is usuallyassociated with pulmonary hypertension and severe symptoms. We discuss treatment of such an infant. Twoabnormal systemic vessels supplying the sequestered lower part of the right lung were embolized usingcatheter-inserted coils. The patent arterial duct was surgically ligated. These procedures resulted in a sig-nificant reduction of the shunt and the level of pulmonary hypertension, as well as in an impresssiveimprovement of symptoms.Key words: Coil embolization, pulmonary hypertension, scimitar syndromeSCIMITAR SYNDROME IS A WELL DESCRIBEDentity, characterised by partially anomalousconnection of the pulmonary veins to theinferior caval vein, hypoplasia of the right lung,right-sided position of the heart and systemicarterial supply from the abdominal aorta to thesequestered segments of the lung, usually in thelower lobe of the right lung.1 Presentation in theneonatal period or in infancy is rare, but thensymptoms are usually severe with the prognosisbeing related to the associated cardiovascularanomalies. In older children, symptoms are lesssevere and the syndrome is usually detected wheninvestigation is undertaken for recurrentpulmonary infections, chest x-ray abnormalities ora heart murmur. We discuss presentation of thesyndrome in a neonate with severe symptoms.Coil embolization of the abnormal systemic arter-ial supply to the sequestered pulmonary segments,together with surgical closure of the arterial ductresulted in a favourable outcome.Correspondence to: Dr Jean-Pierre Pfammatter, MD. Division of PediatncCardiology Children's Hospital, Freiburgstrasse, CH-3010 Berne, Switzerland.Fax: ++41 31 632 9748Accepted for publication 7 June 1996Case reportThis boy was born after an uneventful pregnancyat 40 weeks of gestation as the second child tohealthy parents. Family history did not revealcardiopulmonary anomalies, except for frequentrecurrence of pulmonary airway obstructionsnecessitating inhalative bronchodilatatory treat-ment in the older brother. In that brother, a right-sided heart was found as an incidental finding lateron. He underwent cardiac catheterization andcardiovascular malformations could be excluded.The patient reported was found to have a right-sided heart at a routine examination at three daysof age. Investigation in another hospital led to thesuspicion of scimitar syndrome, and the patientwas sent to our centre for evaluation at the ageof three weeks. On admission, the boy wastachypneic with almost silent pulmonary ausculta-tion on the right side in comparison to normalauscultatory findings on the left side. Heart soundswere loudest at the right sternal border. There wasa single and loud second heart sound, as well as agrade II/VI systolic murmur. Heart rate was166/minute. The liver was enlarged. Chest X-rayshowed deviation of the heart and themediastinum to the right side of the thorax, severehttps:/www.cambridge.org/core/terms. https://doi.org/10.1017/S1047951100004509Downloaded from https:/www.cambridge.org/core. University of Basel Library, on 30 May 2017 at 19:48:10, subject to the Cambridge Core terms of use, available atVol. 7, No. 4 Pfammatter et al: Coli embolization in infantile scimtar syndrome 455Figure 1.Two systemic vessels originating from the abdominal aorta andsupplying a large area of the right lung are seen; the proximalvessel arises at the level of the diaphragm, the more distal vesselarises near the celiac artery.hypoplasia of the right lung, and compensatoryexpansion of the left lung. Vascular markings onthe left side were increased. The electrocardiogramshowed prominent P-waves in the right precordialleads and signs of right ventricular hypertrophy.Echocardiography showed usual atrial arrange-ment, an atrial septal defect with left-to-rightshunt, patency of the arterial duct with a smallleft-to-right shunt, an enlarged right atrium andright ventricular hypertrophy. The left-sidedpulmonary veins were shown to drain into the leftatrium, whereas the right-sided pulmonary veinsconnected into the inferior caval vein. There wasevidence of severe pulmonary hypertension.Pulmonary perfusion scintigraphy revealed aperfusion imbalance, with a left/right ratio of85/15%.Cardiac catheterization was performed at the ageof 6 weeks. The standard femoral venous approachwas used, with the patient under general anesthe-sia. Oxygen saturation in the superior caval veinwas 58%, in the right atrium 83%, and in theinferior caval vein 91%- Systolic pulmonary pres-sure was at systemic level (67 mmHg, with thesystolic left ventricular pressure being 70 mmHgat the same time). The left-to-right shunt at atriallevel was calculated to be 56%, and the pulmonaryresistance was elevated to 4 Woods units.Angiography revealed an atrial septal defect,patency of the arterial duct, right-sided heart,partially anomalous pulmonary venous connectionfrom the entire right lung via a vertically descend-ing (scimitar) vein into the inferior caval veinjust below the right atrium. Additionally, twoabnormal arteries originating from the abdominalaorta were identified supplying a large sequesteredarea in the lower part of the right lung (Fig. 1). Asthese arteries were considered to addsignificantly to the total left-to-right shunting andto pulmonary hypertension, it was decided toocclude them. The distal vessel (diameter 2.5 mm)Table 1: Comparison of catheterization dataParameters atcatheterizationPulmonary arterial pressureSystemic pressureRatio pulmonary/systemic pressurePVR (Woods units)Left-right shuntPre-embolization67/19 (mean 37)70/23 (mean 38)1456%Post-embolization52/22 (mean 3D76/34 (mean 42)0.7not assessednot assessed8 monthsafter embolization40/12 (mean 25)96/59 (mean 73)0.4236%(PVR = pulmonary vascular resistance, LV = left ventricle)https:/www.cambridge.org/core/terms. https://doi.org/10.1017/S1047951100004509Downloaded from https:/www.cambridge.org/core. University of Basel Library, on 30 May 2017 at 19:48:10, subject to the Cambridge Core terms of use, available at456 Cardiology in the Young October 1997was occluded first using two steel coils with ahelical diameter of 3 mm (MWCE 38-4-3, CookEurope Inc). The more proximal vessel (diameter3,5 mm) was then occluded with three coils, thefirst two having a coiled diameter of 5 mm(MWCE 38-5-5) and the last one having a diame-ter of 3 mm. Angiography after 15 minutesrevealed complete occlusion of both vessels withno residual flow of contrast (Fig. 2). Systolicpulmonary arterial pressure had dropped to about3/4 of systemic pressure (Table 1). The boy was leftintubated and ventilated, and treatment withprostacyclin (epoprostenol) infusion was started inorder further to decrease pulmonary resistance toallow surgical ligation of the duct. This was per-formed without complications 6 days after theinterventional catheterization. The patient recov-ered well but needed further treatment consistingof oral and inhalative bronchodilatatory agents forfrequently recurrent pulmonary airway obstruc-tion.Repeat catheterization was performed at theage of 9 months. The child was still on bronchodi-latatory treatment, but otherwise had thrivedwell and had no symptoms except for a slightlyFigure 2.Selective arteriography of the more proximal of the two abnormalsystemic vessels shows no residual flow after placement of the coils.More distally, the second artery is shown with the coils alreadyin place. No flow of contrast is seen.elevated respiration rate. Catheterization revealedresidual left-to-right shunting at atrial level(36%). Systolic pulmonary arterial pressure was 40mmHg (mean 24), with the left ventricularsystolic pressure at the same time being at 96mmHg (Table 1). Pulmonary vascular resistancewas calculated to be 2 Woods units.In the lack of severe symptoms, and because ofthe clinically stable condition, no further invasivetreatment was deemed necessary. At the lastfollow-up visit (March 1995, 19 months after theintervention), the child was in good health. Therewas no history of pulmonary infections, the patientwas still on prophylactic inhalative bronchodilata-tory treatment but he had suffered no furtherepisodes of acute pulmonary airway obstructions.The electrocardiogram showed signs of mild rightventricular hypertrophy. Follow-up examinationsare planned at yearly intervals.DiscussionScimitar syndrome is a distinct cardiovascularand pulmonary malformation.2 Clinically, thereare two modes of presentation: in the older childthe syndrome usually manifests as recurrentpulmonary infections or as a heart murmur. Inthese patients impressive symptoms are rarelyfound, and prognosis is generally good.3 On theother hand, the variant encountered in infancy isassociated with marked pulmonary hypertension4and is usually associated with severe symptoms ofrespiratory distress, heart failure, or even cyanosis.In combination with other cardiovascular malfor-mations, the prognosis is poor even when surgicalor interventional therapy is undertaken.4'5Symptomatic infants with the syndrome arecharacterised by the presence of usually severepulmonary hypertension with systemic orsuprasystemic pulmonary arterial pressures.5Pulmonary hypertension is found in these patientsin the presence of small shunts which, in othercircumstances, do not cause pulmonary hyperten-sion.3 It has been hypothesized that the abnormalsystemic flow to the sequestered area of the rightlower lung, which is typically found in theinfantile form of the syndrome, contributedsignificantly to the severity of symptoms and topulmonary hypertension.4' 6 Histologic examina-tion of the pulmonary vascular structuresin infants with scimitar syndrome revealed anabnormally increased muscularity of pre- andintraacinar pulmonary arteries in both lungs.7Treatment of the scimitar syndrome, when seenin infancy, is directed at reducing the shunt andhttps:/www.cambridge.org/core/terms. https://doi.org/10.1017/S1047951100004509Downloaded from https:/www.cambridge.org/core. University of Basel Library, on 30 May 2017 at 19:48:10, subject to the Cambridge Core terms of use, available atVol. 7, No. 4 Pfammatter et ai. Coli embolization in infantile scimtar syndrome 457decreasing pulmonary hypertension. This seems tobe best achieved by interruption of the abnormalsystemic arterial supply from the abdominal aortato the sequestered segments of the right lung,together with closure of the usually associatedshunt lesions, such as an atrial septal defect or apatent arterial duct. Standard therapy has beensurgical. This treatment is associated with consid-erable morbidity and mortality.5 Lobectomy andpneumonectomy have also been performed.3'4Advances in interventional catheterization havenow made it possible effectively to occludeabnormal intrathoracic vessels.8 As early as in1982, this method was advocated for thetreatment of scimitar syndrome in infancy. In thisearly experience embolization using Gelfoamfragments was unsuccessfully attempted in twoinfants but was successful in one.6 To the best ofour knowledge, only four infants with scimitarsyndrome who have undergone successfulembolization have been reported so far. One largeexperience on the use of coil embolization inchildren included one child with scimitarsyndrome in whom successful embolization hadbeen performed but the age of the patient was notspecified.9 In another study, embolization wasattempted in four infants with scimitar syndrome(using coils in two cases, a detachable balloon andtissue adhesive in one patient, respectively) andwas successful in two.5In our patient, the embolization of the abnormalvessels supplying a large sequestered area in thelower lobe of the right lung resulted in a markedreduction of pulmonary arterial pressure and in asignificant reduction of the shunt volume. Thecontribution of surgical ligation of the duct isdifficult to assess. Based on the experience ofothers,4 it might well have been of minorimportance. The favorable outcome shows thatearly interruption of systemic flow throughpulmonary sequestrations is a promising therapeu-tic option to decrease the shunt volume andameliorate pulmonary hypertension. Transcatheterembolization cannot be compared to results ofsurgical ligation, as only very few infants with theformer approach have been reported. Ourexperience, nonetheless, shows that coil emboliza-tion can be successfully carried out even in smalland sick infants. It should probably be consideredas the optimal mode of treatment in infants withsymptomatic scimitar syndrome. As with all inter-ventions in this disease, it remains palliative innature.References1. Fisher DR, Zuberbiihler JR. Pulmonary venousabnormalities. In: Anderson RH, Macartney FJ, ShinebourneEA, Tynan M. Pediatnc Cardiology. Churchill Livingston,Edinburgh 1990; vol I, p.5092. Neill CA, Ferencz C, Sabiston DC, Sheldon H. The familialoccurrence of hyppoplastic right lung with systemic arterialsupply and venous drainage' Scimitar syndrome. Bulletin ofthej Hopkins Hospital I960; 107: 1-213. Canter CE, Martin TC, Spray TL, Weldon CS, Strauss AW.Scimitar syndrome in infancy. Am J Cardiol 1986; 58:852-8544. Dupuis C, Charaf LAC, Breviere GM, Abou P. Infantile formof the scimitar syndrome with pulmonary hypertension. AmJ Cardiol 1993; 71: 326-3305. Gao YA, Burrows PE, Benson LE, Rabinovitch M, FreedomRM. Scimitar syndrome in. infancy. J Am Coll Cardiol 1993;22: 873-8826. Dickinson DF, Galloway RW, Massey R, Sankey R, Arnold R.Scimitar syndrome in infancy. Role of embolization ofsystemic arterial supply to right lung. Br Heart J 1982; 47:468-4727. Haworth SG, Sauer U, Biihlmeyer K. Pulmonaryhypertension in scimitar syndrome in infancy. Br Heart J1983; 50: 182-1898. Reidy JF, Jones ODH, Tynan M, Baker EJ, Joseph MC.Embolisation procedures in congenital heart disease. BrHeart J 1985; 54: 184-1929. Perry SB, Radtke W, Fellows KE, Keane JF, Lock JE. Coilembolization to occlude aortopulmonary collateral vesselsand shunts in patients with congenital heart disease. J AmColl Cardiol 1989; 13: 100-108https:/www.cambridge.org/core/terms. https://doi.org/10.1017/S1047951100004509Downloaded from https:/www.cambridge.org/core. University of Basel Library, on 30 May 2017 at 19:48:10, subject to the Cambridge Core terms of use, available at

Infantile scimitar syndrome with severe pulmonary hypertension: successful treatment with coil embolization of the systemic arterial supply to the sequestered lung

Infantile scimitar syndrome with severe pulmonary hypertension: successful treatment with coil embolization of the systemic arterial supply to the sequestered lung

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