Surgical Correction of Pulmonary Atresia with Ventricular Septal Defect and No Central Pulmonary -Arteries Sulekha Kumar, MD, Daniel Scagliotti, MD, Elizabeth A. Fisher, MD, and Pedro del Nido, MD s recently as 1988 patients with pulmonary atresia with ventricA ular septal defect and no central pulmonary arteries were dismissed as having “no prospect of complete repair.“’ We report our experience in the management and successful staged repair of this anomaly in a 4%-year-old child. PS was cyanotic at birth and diagnosed as having pulmonary atresia with ventricular septal defect and no central pulmonary arteries. All 19 bronchopulmonary segments were perfused by collateral arteries and a patent ductus arteriosus (Figures IA, B, and 2A). A 5-mm Gortex graft was placed from the left innominate artery to the confluence of the left lobar pulmonary arteries (Figures ID and 2B). At 3 years of age, angiography showed afunctioningshunt and 2 stenotic aortopulmonary collateral arteries, which provided the major blood supply to the right lung. Pressure distal to the stenosis was 28118 mm Hg, and aortic oxygen saturation was 73%. A 6-mm Gortex graft was placed from the right subclavian artery to right lower and upper lobe collateral arteries as they entered the lung parenchyma (Figures I C and 2B). At 4 years of age, an angiogram showed that both shunts were functioning. The aortic oxygen saturation was 82%, and the ratio of pulmonary to systemic blood flow (QP/QS) was I .4/l. Fourteen bronchopulmonary segments were perfused via the shunts. An additional large pulmonary artery supplied a portion of the right upper lobe. At surgery, this artery was identiJied in the fissure between the upper and middle lobes. A lo-

mm Gortex graft was attached to it and to the previous 6-mm graft (Figure 2C). A 20-mm aortic homograft was used to connect the right ventricle to the lobar pulmonary arteries on the left. A side arm of 12-mm Gortex graft connected the homograft to the lo-mm graft on the right

20.

?his report adds another patient to 2 previously reported2 who have undergone successfulrepair of pulmonary atresia with ventricular septal defect and no central pulmonary arteries. All 3 had the majority of bronchopulmonary segments perfused by systemic arteries and large

FIGURE J.SekctZveeontra~ngiography,frontalviews.A,preoperativerSgM-

slded aerlm From the College of Medicine at Chicago, Division of Pediatric Cardiology, the University of Illinois at Chicago, 840 South Wood Street, Chicago, Illinois 606 12. Manuscript received June 12, 1989; revised manuscript received September 18,1989, and accepted September

(Figures 2D and 3). The ventricular septal defect was closed and the systemic-pulmonary shunts were ligated. Postoperatively, right/left ventricular systolic pressure ratio was 0.45. There was a small residual ventricular septal defect. The patient is doing well 19 months postoperativelv.

(c) lnjec6en. Saferal areas ef stenesls are and the correrponcling lateral, 10 konchopulmonaryMwereFB,wtieleftpulmonary

ideated

(amws).

dated

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artety injectbn via the patent ducftrs adenoid (PDA). Nine konchopuknpMv segnleh were illedhd. c, lighl Gerlex grafl injec6lnl after lal-. lnwhqdmenary segmenb were seen. D, left Gedex grafl injectlon. E&l konchoplknoMIy~were~.65=5mmOortexgraft;66=6mm Gerlex grae IA = innominate artery.

THE AMERICAN JOURNAL OF CARDIOLOGY JANUARY 15, 1990

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lobar pulmonary arteries accessible for unifocalization. In our patient, 14 of 19 bronchopulmonary segments could be identified beforerepair. The first patient of Barbero-Marcia1 et al2 had 4 aortopulmonary collateral arteries and at least 16 of 19 bron-

chopulmonary segments were perfused. Their second patient had 3 aortopulmonary collateral arteries perfusing all 19 bronchopulmonary segments.Our patient had high aortic oxygen saturation and QP/QS, evidence that the lobar pulmonary

arteries were of adequatesize for repair.3 The normal pressure in the right lower lobe pulmonary artery was evidenceagainst pulmonary vascular disease.The first patient reported by Barbero-Marcia1 et al2 presentedat 18 months of age with

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43

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(H)wos-~to~ -ofbborpmonrlr~ ontheb#t.Asi&mnofl2-mmGortox grolt (Gl2) was ussd to connect

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THE AMERICAN JOURNAL OF CARDIOLOGY VOLUME 65

congestiveheart failure, evidencefor increasedpulmonary blood flow and large pulmonary arteries. In all 3 patients a staged surgical approach replaced systemic collateral arteries with grafts to supply adequate but controlled perfusion, promotegrowth of the pulmonary arteries and protect against pulmonary vascular disease. Unifocalization simplified establishing perfusion from the newly constructed right ventricular outflow

A Transmural Approach Ventricular Pacing

tract at repair. Angiography between procedureshelped refine the surgical approach.By utilizing the newly created aortopulmonary connections,it was possibleto define the sourcesof pulmonary perfusion better than at the initial study. Postoperativeevaluation showedperfusion of the majority of bronchopulmonary segments and acceptableright/left ventricular systolic pressureratios. The clinical results were good in all 3 patients.

for Endocardial

thoracotomy and endocardial leads placed transvenously. At times, the usual transvenousapproachesare not feasible, such as in superior vena cava obstruction or impaired access to the right ventricle becauseof tricuspid stenosisor a mechanical prosthetic valve. Usually, such situations necessitateepicardial pacing, which sometimesis unsatisfactory because of poor thresholds or inadequate sensing with epicardial leads. We present a casein which a new method for endocardial ventricular lead placement was usedwhen both standard endocardial pacing and epicardial pacing were unsatisfactory.

The patient was asymptomatic over the following 4 years.

.,j

‘i

Thorar Cardiouasc Surg 1987,94:91 l-914.

3. Millikan JS, Puga FJ, DanielsonGK, Schaff HV, Julsrud PR, Mair DD. Stagedsurgical repair of pulmonary atresia, ventricular septal defect, and hypeplastic,confluent pulmonary arteries. J Thorac Cardiocasc Surg 1986:91:818-825.

In 1988, the patient presented with progressive dyspnea and fatigue. Electrocardiographic and pacemaker analysis revealed complete heart block with a ventricular rate of 30 beats/min and failure to capture despite maximal energy settings (8.1 Vat 0.92-mspulse width).

Benjamin D. McCallister, Jr., MD, Ronald E. Vlietstra, MB, chB, Benjamin M. Westbrook, MD, and David L. Hayes, MD he 2 standard approaches to T placementof cardiac pacemaker leads are epicardial leads applied at

1. Sullivan ID, Wren C, Stard J, de Leval MR, Macartney FJ, Deanfield JE. Surgical unifocalization in pulmonary atresia and ventricular septal defect. Circulation 1988:(suppl 11l)78:111-5-111-13. 2. Barber@Marcia1M, Rizzo A, Lopes AAB, Bittencourt D, Junior JOA, Jatene AD. Correction of pulmonary atresia with ventricular septal defect in the absenceof the pulmonary trunk and the central pulmonary arteries (so-called truncus type IV). J

‘(

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A 63-year-old woman had StarrEdwards prosthetic valves in aortic, mitral and tricuspid positions. At age 59 she had developed complete heart block with a ventricular escape rate of 30 beatslmin that required epicardial lead placement because of the prosthetic tricuspid valve. Positioning of the lead was difficult because of high thresholds. Duringfollow-up, thepatient developedfailure to capture, but her intrinsic heart rate had increased to 50 beats/min. From the Division of Cardiovascular Diseases and Internal Medicine and the Division of Thoracic and Cardiovascular Surgery, Mayo Clinic and Mayo Foundation, 200 First Street SW, Rochester, Minnesota 55905. Manuscript received April 24, 1989; revised manuscript received and accepted September 1, 1989.

flGURE 1. Transmwal ventrkular had phement. Ventricular emhwardial bad is passodthroughrigM-wauandactivolyfixatedtorightvOlUbCaNiiUIll.Aprre~Iubre sesames lead in lighl ventrkular free wall. (By porddon of Mayo Foundation.) THE AMERICAN JOURNAL OF CARDIOLOGY JANUARY 15, 1990

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