Definitive Repair of Double-Outlet Right VentricleWith Subaortic Ventricular Septal Defect and

Pulmonary Atresia in Adulthood

Scott A. McKee, MD, Charles S. Roberts, MD, and Efthymios N. Deliargyris, MD

Definitive surgical repair ofdouble-outlet right ventricle

(DORV) is usually done early inlife (�6 months), with 10-year sur-vival rates as high as 90%.1,2 Cor-rective surgery later in life (after anearly palliative operation) is notusually an option due to the pres-ence of significant pulmonary hy-pertension and right ventriculardysfunction. Two reported patientswith repair after the age of 19 diedwithin 36 months.3,4 We present ayoung man with 2 previous pallia-tive operations, who at 21 years ofage, underwent repair of DORVwith a subaortic ventricular septaldefect and pulmonary atresia.

• • •A.B., a 21-year-old Caucasian

male, presented to the Universityof North Carolina Hospitals withlight-headedness, dysphagia, andleft arm weakness. A right Bla-lock-Taussig anastomosis had beenperformed shortly after birth, andan aorto-pulmonary shunt wasdone at the age of 4 years. Dyspneaduring exertion had worsened inrecent months. Peripheral oxygensaturation was 85%. The pulmo-nary component of the secondheart sound was absent; a fourthheart sound and a harsh grade 4/6continuous murmur was present atthe second left intercostal space.The distal fingers and toes wereclubbed. The hematocrit was 63%.An electrocardiogram showed si-nus rhythm with right ventricularhypertrophy and bifascicular

block. The results of a computedtomogram of the head were nega-tive. Transthoracic echocardiogra-phy demonstrated an overridingaorta with a large ventricular septaldefect, mild left ventricular dys-function, right ventricular hyper-trophy with mild dysfunction, pul-monary atresia, and mild aortic re-gurgitation. The patient was treatedwith phlebotomy.

Right- and left-sided heart cath-eterization and ventriculographyconfirmed a large ventricular septal

defect, an atretic pulmonary artery,and equalization of pressures be-tween the left and right ventricles(Figure 1). The 2 shunts were se-lectively cannulated. The right andleft pulmonary artery pressureswere normal (Table 1) with signif-icant narrowing at both sites (Fig-ure 2). The left anterior descendingcoronary artery arose from the leftcoronary cusp, and a single arteryarising between the right and pos-terior cusps divided into the rightand left circumflex arteries.

From the Cardiology Section, Department ofMedicine, Wake Forest University School ofMedicine, Winston-Salem, North Carolina;and Division of Thoracic and CardiovascularSurgery, Winchester Surgical Clinic, Win-chester, Virginia. Dr. Deliargyris’ address is:Cardiology Section, Wake Forest UniversitySchool of Medicine, Medical Center Boule-vard, Winston-Salem, North Carolina 27157-1045. E-mail: edeliarg@wfubmc.edu.Manuscript received August 29, 2002; re-vised manuscript received and acceptedSeptember 18, 2002.

FIGURE 1. Left ventriculography from the left anterior oblique projection demonstratinga large subaortic ventricular septal defect (white arrow) is shown. The arrowhead indi-cates the pigtail catheter transcending the ventricular septal defect and ending up in theRV. The black arrow indicates the pulmonary artery catheter traveling from the RVthrough the ventricular septal defect into the ascending aorta. LV � left ventricle; RV �right ventricle.

TABLE 1 Preoperative Pressure and Oxygen Saturation Measurements

Pressures(mean) (mm Hg)

OxygenSaturation (%)

RA 8/3 (4) 59RV 130/16 84Right pulmonary artery 17/7 (11) 84Left pulmonary artery 15/8 (12) 84Left ventricle 130/16 94Systemic arterial 130/60 (91) 85

380 ©2003 by Excerpta Medica, Inc. All rights reserved. 0002-9149/03/$–see front matterThe American Journal of Cardiology Vol. 91 February 1, 2003 doi:10.1016/S0002-9149(02)03179-X

A month later the patient pre-sented to the emergency room witha high fever (103°F), chills, sorethroat, and diarrhea. Blood cultureswere positive for oxacillin-sensi-tive Staphylococcus aureus, andthe patient was treated with a pro-longed course of intravenous anti-biotics for presumed infective en-docarditis. Eight weeks later he re-turned for his elective operation.The ventricular septal defect wasclosed with a Dacron (Dupont,Kinston, North Carolina) patchcreating a left ventricular outflowtract to the aorta. The right ventric-ular outflow tract was recon-structed with a homograft conduit.

The 2 preexisting shunts were li-gated. The sternum was closed theday after. A repeat echocardiogrambefore discharge showed mildbiventricular dysfunction and mildtricuspid and aortic regurgitation.The patient was discharged homeon digoxin, atenolol, and aspirin.

The patient reported completeresolution of his exertional dys-pnea during the 8-week follow-upvisit. His oxygen saturation was95% without decrease with moder-ate exercise and all peripheral club-bing had resolved. The hematocritwas 49%. Currently, the patient is30 months out from his operationand living a normal, active life

without physical limitations. He iscompleting his undergraduate stud-ies in marine biology and since theoperation he has become an avidand certified S.C.U.B.A. diver(Figure 3).

• • •This case suggests that under

the right physiologic circum-stances, late repair can be success-ful and improve the patient’s qual-ity of life.4,5 There were 2 impor-tant features that can explain thefavorable clinical outcome in ourpatient. First, the presence of nor-mal pulmonary artery pressures.The 2 palliative shunts were placedat an early age and as the patientgrew they became relatively under-sized. The subsequent shunt/pul-monary artery size mismatch wasfunctioning as a hemodynamicallysignificant stenosis, shielding thepulmonary circulation from sys-temic pressures. In addition, thepulmonary circulation was pro-tected from the systemic right ven-tricular pressures by the atretic pul-monary valve. The second impor-tant feature in this case was thepresence of preserved left and rightventricular systolic function preop-eratively. The high mortality en-countered in patients who undergolate repair is related to pump fail-ure postoperatively, which sug-gests that preserved biventricularfunction is necessary to sustain thehemodynamic stress of surgicallyimposed normalization of systemicand pulmonary circulation.5,6

In summary, definitive repairof DORV can be undertaken inadulthood with an excellent out-come. In our patient, 2 previouspalliative shunts in childhoodhad not resulted in ventriculardysfunction or pulmonary hy-pertension, which conferred a fa-vorable surgical risk.

1. Walters HL, Mavroudis C, Tchervenkov CI, Ja-cobs JP, Lacour-Gayet F, Jacobs ML. Congenitalheart surgery nomenclature and database project:double-outlet right ventricle. Ann Thorac Surg 2000;69(suppl 4):S249–S263.2. Takeuchi K, McGowan FX, Moran AM, Zura-kowski D, Mayer JE, Jonas RA, del Nido PJ. Surgi-cal outcome of double-outlet right ventricle withsubpulmonary VSD. Ann Thorac Surg 2001;71:49–53.

FIGURE 2. Left panel, an ascending aortogram with significant narrowing at the aorto-pulmonary shunt site (second palliative operation) is shown. Right panel, a selective an-giogram of the Blalock-Taussig shunt with significant narrowing at the shunt site (firstpalliative operation) is shown. Arrows depict stenosis at the anastomotic site.

FIGURE 3. A.B. diving with friends off the Cozumel coast of Mexico.

CASE REPORT 381

3. Aoki M, Forbess JM, Jonas RA, Mayer JE, Cas-taneda AR. Result of biventricular repair for double-outlet right ventricle. J Thorac Cardiovasc Surg1994;107:338–350.4. Belli E, Serraf A, Lacour-Gayet F, Prodan S, PiotD, Losay J, Petit J, Bruniaux J, Planche C. Biven-

tricular repair for double-outlet right ventricle: re-sults and long-term follow-up. Circulation 1998;98(suppl 19):II-360–II-367.5. Perloff JK, Warnes CA. Challenges posed byadults with repaired congenital heart disease. Circu-lation 2001;103:2637–2643.

6. Tchervenkov CI, Pelletier M, Shum-Tim D, Be-land M, Rohlicek C. Primary repair minimizing theuse of conduits in neonates and infants with tetralogyor double-outlet right ventricle and anomalous cor-onary arteries. J Thorac Cardiovasc Surg 2000;119:314–323.

382 THE AMERICAN JOURNAL OF CARDIOLOGY� VOL. 91 FEBRUARY 1, 2003