Variant of tetralogy of fallot with absent pulmonary valve leaflets and origin of one pulmonary artery from the ascending aorta

PEDIATRIC CARDIOLOGY

Variant of Tetralogy of Fallot With Absent Pulmonary Valve Leaflets and Origin of One Pulmonary Artery From The Ascending Aorta

A. LOUISE CALDER, MD PETER W. T. BRANDT, MB, ChB, FRCR, FRACR BRIAN G. BARRATT-BOYES, MB, Chtvl, FRACS JOHN M. NEUTZE, MD, FRACP Auckland,

New Zealand

Absent pulmonary valve leaflets and anomalous origin of a pulmonary artery from the ascending aorta have been reported as separate lesions associated with tetralogy of Fallot but not in comblnatlon. The clinical, radlologic and pathologic ftndings are presented in three patients with this previously undescrlbed combination. A search of the literature for similar cases revealed 233 patients with absent pulmonary valve leaflets and 56 patients with anomalous origin of a pulmonary artery from the ascendlng aorta; these cases are reviewed. Although 17 patients with absent pulmonary valve leaflets and tetrakgy of Fallot also had absence of the proximal portion of the left pulmonary artery, none had an anomalously arising pulmonary artery from the ascending aorta. In the reviewed cases, anomalous origin of the right pulmonary artery from the ascending aorta was more frequent than that of the left pulmonary artery (44 compared with 12 cases, respectively), but the latter was more often associated with tetralogy of Fallot.

Tetralogy of Fallot has been associated with several congenital cardiac lesions, including absent pulmonary valve leaflets, or with origin of either the right or left pulmonary artery from the ascending aorta. However, the combination of these defects with tetralogy of Fallot has not been previously reported, as far as we know. Absence of the pulmonary valve leaflets has been reported in 233 cases. l-s1 Anomalous origin of the left pulmonary artery from the ascending aorta was reported in 12 other cases82-g1 and of the right pulmonary artery in 44 cases.17,s4,ss,ss,90,92~ 119

The purpose of this paper is to present three patients with tetralogy of Fallot who had the absent pulmonary valve leaflet syndrome, resulting in dilation of the pulmonary arteries, which produced bronchial compression. In addition, the left pulmonary artery in two of the patients and the right pulmonary artery in the third arose from the ascending aorta. Features

From the Green Lane Hospital, Auckland, New Zealand. Manuscript received November 7, 1979; revised manuscript received December 28, 1979, accepted January 11, 1980.

Address for reprints: A. Louise Calder, MD, Cardiology Department, Green Lane Hospital. Green Lane West. Auckland3, New Zealand.

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Clinical findings: The three patients presented at Green Lane Hospital between 1964 and 1974 with congestive heart failure, failure to thrive and mild cyanosis. Cyanosis had been noted with crying in Patient 1 at age 1 week; he also had a cleft soft palate. Although Patient 2 was delivered after an apparently normal pregnancy, her birth weight was ‘2.4 kg; only a single umbilical artery was noted. A heart murmur was heard during a routine examination at age 6 weeks.

Systolic and diastolic murmurs were noted shortly after birth in the examination of Patient 3, who exhibited cyanosis and breathlessness at age 3 months. Patients 2 and 3 had frequent respiratory infections.

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FIGURE 1. Chest roentgenograms of Patient 1 (anomalous origin of left pulmonary artery) at age 4 days (a) showing cardiomegaly and pulmonary plethora on the left and oligemia on the right, and at age 1 month (b) showing increased cardiomegaly and partial collapse of the right lung with right upper lobe consolidation. The asymmetry of pulmonary vasculature persists.

Physical examination revealed full and jerky pulses in the three patients together with continuous or ejection and early diastolic murmurs. Patient 1 died from “inhalation pneumonia” 4 hours after admission to hospital at age 1 month. Patients 2 and 3 were admitted to the hospital at 2 and 3 months of age, respectively, and their diagnoses were established with cardiac catheterization and cineangiography. Patient 2 made reasonable progress after discharge from hospital until age 8 l/2 months, when she died suddenly at home after a “chest cold.” Patient 3 was severely ill and required assisted ventilation because of respiratory distress due to bronchiolitis and pneumonia before surgical correction was undertaken. The repair was performed utilizing profound hypothermia1zo at age 4 months. The right pulmonary artery was detached from the ascending aorta and anastomosed to the main pulmonary artery. The ventricular septal defect was closed with a Teflon@ patch and a portion of the subpulmonary conal muscle excised. The right ventricular outflow tract incision was carried across the pulmonary ring to the left pulmonary artery and a valved aortic homograft conduit inserted. The patient died 36 hours postoperatively with low cardiac output and severe pulmonary infection. Chest roentgenograms: Cardiomegaly was present in all three patients (Fig. 1 and 2). The lung vasculature was asymmetric, with increased perfusion on the side supplied by the anomalously arising pulmonary artery (Fig. 1 and 2). The aneurysmally dilated right or left pulmonary arteries were visible in Patients 2 and 3 (Fig. 2, a and b). Electrocardiograms: The electrocardiograms in all three patients showed right axis deviation (+ 120”) and combined ventricular hypertrophy. Cardiac catheterization: Cardiac catheterization studies performed in Patients 2 and 3 revealed arterial desaturation (71 and 83 percent, respectively). A gradient of 38 and 41 mm Hg, respectively, was present across the right ventricular outflow tracts. The right ventricular systolic pressure was at systemic levels. In Patient 2 the anomalously arising left pulmonary artery was not entered from the ascending aorta. In Patient 3 a gradient of 7 mm Hg was noted across the origin

of the right pulmonary artery, which arose from the ascending aorta. Cineangiocardiographic findings: The cineangiocardiograms in Patients 2 and 3 showed narrowed pulmonary rings with no evidence of pulmonary valve leaflets (Fig. 3a and 4, a and b). The right pulmonary artery in Patient 2 and the left pulmonary artery in Patient 3 were severely dilated. The conal septum was deficient in Patient 2 (Fig. 3a), whereas displacement and hypertrophy of the conal septum produced infundibular narrowing in Case 3 (Fig. 4a). The origin and proximal portion of the left pulmonary artery, which arose from the ascending aorta, was narrowed in Patient 2 (Fig. 3b) and the origin of the anomalous right pulmonary artery was mildly narrowed in Patient 3 (Fig. 4 c and d). Anatomic findings: The pertinent necropsy findings were confined to the heart arteries and great arteries. The three patients had situs soiitus of the atria, ventricular d-loops, normally related great arteries and findings compatible with a diagnosis of tetralogy of Fallot (Fig. 5 to 7). The aortic, mitral and tricuspid valves and the coronary arteries were normal. A subaortic, moderate-sized ventricular septal defect was present above the septal bandizl (Fig. 5 and 6). In Patient 2 the conal septum was markedly deficient and measured only 1.5 mm from the pulmonary ring to its lower edge (Fig. 6, b and c). The poorly expanded right ventricular outflow tract measured 5 by 7 mm in internal diameter. Although the aortic valve overrode the right ventricular cavity, there was direct fibrous continuity between the anterior leaflet of the mitral valve and the left coronary and noncoronary aortic valve leaflets. Subpulmonary infundibular narrowing was present in Patients 1 and 3 because of anterior and leftward displacement of the conal septal end of the parietal band (Fig. 5b). In Patient 1 deficiency of the right posterior division of the septal band allowed the ventricular septal defect to extend to the tricuspid valve. The pulmonary valve leaflets were absent. A narrow, fibrous ridge or small nubbins of fibrous tissue were present in the narrowed pulmonary rings, which measured 7,9 and 11 mm, respectively, in external diameter. (Fig. 5b, 6, b and c, and

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FIGURE 2. a, Patient 2 (anomalous origin of left pulmonary artery). Chest roentgenogram taken at 2 months of age shows cardiomegaly, markedly enlarged right pulmonary artery with oligemia peripherally and collapse of the right upper lobe. Left pulmonary vasculature seems normal. b, Patient 3 (anomalous origin of right pulmonary artery). Chest roentgenogram at 11 weeks of age shows a markedly dilated left pulmonary artery with left peripheral oligemia and right pulmonary plethora

7). The rings were 2 to 3 mm above the junction of the right ventricle with the pulmonary trunk. The pulmonary trunks were relatively hypoplastic and then expanded into a single, aneurysmally dilated pulmonary artery: The right pulmonary artery in Patients 1 and 2 (external diameter 13 and 20 mm, respectively) and the left pulmonary artery in Patient 3 (external diameter 23 mm) (Fig. 5b, 6c and 7). The anomalous left pulmonary artery arose from the ascending aorta 6 and 7 mm above the aortic valves in Patients 1 and 2, respectively. The narrowed ostium of the left pulmonary artery in Patient 2 measured 1.5 to 2 mm in internal diameter and the left pulmonary artery was narrowed for 5

mm beyond its origin (Fig. 3b and 6a). In Patient 1 a fibrous strand extended from the base of the left pulmonary artery to the pulmonary trunk (Fig. 5~). The aortic arch was right-sided in Patient 1 and left-sided in the other two patients. Aberrant subclavian arteries were present in Patients 1 and 2. A ductus arteriosus or ligamentum arteriosum could not be identified at either the aortic or pulmonary arterial ends in any of these cases. The right bronchus was compressed by the markedly dilated right pulmonary artery in Patient 2 and the left main bronchus was compressed by the dilated left pulmonary artery in Patient 3.

__ \\ ‘+ .,. ‘\-_

2

!‘.“‘.

“.

FIGURE 3. Patient 2. Cineangiocardiographic frames. a, right ventricular (RV) injection, right anterior oblique (R.A.O.) projection. Marked hypoplasia of the conal septum (C), a moderate-sized infundibular chamber (lnf.), narrowed pulmonary ring (arrow) and dilated right pulmonary artery (RPA) are seen No pulmonary leaflets can be identified. Some right to left flow through the ventricular septal defect faintly fills the aorta (Ao.). b, ascending aortogram (Ao.) left anterior oblique (L.A.O.) projection (venous catheter through ventricular septal defect). Aortogram identifies the right (rca) and left (Ica) coronary arteries, and the left pulmonary artery (LPA), moderately stenotic proximally, arising from the leftward posterior aspect of the mid ascending aorta. The aortic arch is on the left.

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Histologic pulmonary

findings: Medial hypertrophy of the small arteries was noted in the left lung in Patient 1,

where the ratio of the media to the external diameter of the vessels was 19 percent compared with 6 percent in the right lung. The media-diameter ratios in the two lungs were similar in Patient 2 (right lung 13 percent, left lung 10 percent) and Patient 3 (right lung 16 percent, left lung 19 percent). Intimal cell proliferation was present in some small pulmonary arteries in the right lung in Patient 3, but not in the other patients. Sections of the right pulmonary arteries in Patients 1 and 2 showed the transitional type of elastic fiber configuration122 in association with thin and infrequent elastic fibers in the anomalous left pulmonary arteries. In Patient 2 the stenosis of the anomalously arising left pulmonary artery probably prevented the persistence or development of the “aortic” type of elastic fiber configuration. In Patient 3 the left pulmonary artery showed the transitional type, whereas the anomalously arising right pulmonary artery had the aortic type of elastic fiber configuration. These histologic findings are inconclusive

because the absent pulmonary valve leaflets associated with tetralogy of Fallot alter the results in these patients with an anomalously arising pulmonary artery. Similarly, the origin of one pulmonary artery from the ascending aorta alters the expected findings in patients with absent pulmonary valve leaflets. Histologic study of the lung in the three patients revealed bronchiolitis ranging from mild in Patient 2 to a necrotizing bronchiolitis and bronchopneumonia in Patient 3. Areas of atelectasis were noted in Patients 1 and 2 and some congestion was also present in Patient 2.

Review of the Literature Absent Pulmonary Valve Leaflets Chevers’O in 1848 cited the first case reports of absent pulmonary valve leaflets by Crampton15 in 1830 and Favell** in 1842. Since then, reports of 231 further cases have been published1-s1 and 12 additional cases have been seen at Green

FIGURE 4. Patient 3. Cineangiocardiographic frames. a, right ventricular (RV) injection in right anterior oblique (R.A.O.) projection. The conal septum (c) is slightly hypoplastic and displaced as in tetralogy of Fallot. with some flow through the ventricular septal defect to the aorta (Ao). A dilated infundibular chamber (Inf.) leads to the dilated left pulmonary artery (LPA). b. Left pulmonary arteriogram in left anterior oblique (L.A.O.) projection. The stenotic pulmona& ring (arrow) is well profilecf’betweenthe infundibulum and the dilated left pulmonary artery. No pulmonary leaflets are visible. The right ventricle partly fills by pulmonary regurgitation. c and d, ascending aortogram in R.A.O. (c) and L.A.O. (d), projections. Right pulmonary artery (RPA) arises from the leftward posterior aspect of the mid ascending aorta (Ao). There is a left aortic arch with normally arising brachiocephalic arteries.

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Only six patients (2.4 percent) with absent pulmonary valve leaflets had no associated cardiac anomalies.1g~2g~4g~55~6s Uncommonly associated cardiac defects were secundum type of atrial septal defect in one, ll~sl hypoplasia of the right ventricle in one,25 double outlet right ventricle in three16Js*71 and tricuspid atresia in five.22*41,sThe ventricular septum was intact in only eight cases.11,19,25,29,49,55,68 Tetralogy of Fallot or ventricular septal defects were present in 228 of the patients (93.1 percent) with absent pulmonary valve leaflets: tetralogy of Fallot in 185 (75.5 percent) and ventricular septal defect in 35 (14.3 percent); in 8 cases it was not possible to decide whether tetralogy of Fallot or only a ventricular septal defect was present. Aberrant origin of a pulmonary artery from the ascending aorta was not present with absent pulmonary valve leaflets in any of these 245 patients.l-sl Absent origin of the left pulmonary artery: Seventeen cases of tetralogy of Fallot and absent pulmonary valve leaflets were reported in which the origin of the left pulmonary artery The blood supply was absent.~7,18,31,35,40,43,45-47,5~,58.~9,72.73.8~ to the left lung was probably through a patent ductus arteri-

Lane Hospital (excluding the 3 in the present report). These 12 cases include 10 with tetralogy of Fallot, including 1 with situs inversus and dextrocardia, 1 with tricuspid atresia and 1 with isolated absent pulmonary valve leaflets. We have analyzed these 245 cases, which do not include patients with other types of congenital pulmonary incompetence, or with dysplastic pulmonary valves without dilatation of the pulmonary arteries, or patients whose diagnosis of pulmonary incompetence was based only on clinical findings or pressure data at cardiac catheterization. The diagnosis was established at postmortem examination in 113 cases, at surgery in 75 and with cardiac catheterization and angiography in 54; in 3 patients the method of documentation is unknown.5g74 With respect to sex, this anomaly was rather equally distributed: 91 were male and 81 female in the 172 cases in which gender was stated. The age at death, known in 109 cases, ranged from 30 minutes to 73 years (median 6.5 months; average 59 months). If the surgically treated patients are excluded, the median age at death was 3 months (average 47.9 months).

FIGURE 5. Patient 1. Heart specimen. a, frontal view showing the left pulmonary artery (LPA) arising from the ascending aorta (Ao) and passing anteriorly to the dilated right pulmonary artery (RPA) to reach the left hilum. The aortic arch is right-sided. b. opened right ventricle (RV) showing the ventricular septal defect (VW), the displaced conal septum (CS) producing subpulmonary infundibular stenosis and only fibrous tags of tissue in the narrowed pulmonary ring (Ring). The right pulmonary artery is dilated. c, superior external view with the ascending aorta pulled forward to show the fibrous strand (arrow) that extended from the origin of the left pulmonary artery to the right pulmonary artery. LAA = left atrial appendage; LV = left ventricle: RAA = right atrial appendage: Tr = trachea; TV = tricuspid valve.

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osus connecting to the distal left pulmonary artery in five patien&3L35$57%~and perhaps through pulmonary collateral channels in the others, although this was not always stated. We know of no reported case of absent pulmonary valve leaflets in which the proximal portion of the right pulmonary artery was also absent. Surgical management: The variety of surgical procedures utilized (excluding those from Green Lane Hospital) are listed in Table I. The majority of the palliative procedures were performed in infants under 2 years of age and the mortality rate was 69 percent. Corrective procedures were performed in 85 patients. Four (66 percent) of the six patients less than 1 year of age died compared with 32 percent of the patients over 2 years of age. A valve was inserted yn the pulmonary anulus in 16 patients (12.5 percent mortality rate) who were over 3.5 years of age at the time of operation (Table I). An aortic homograft valve was used in six11,37,50,7sand a glutaraldehyde-preserved por-

tine aortic valve in four.14~44~47*72 The type of valve was not stated in six.sa Seven patients with absent pulmonary valve leaflets and tetralogy of Fallot underwent surgery at Green Lane Hospital. Two patients, aged 7 and 14 months, respectively, died

in the hospital. One had a patch tract inserted across the pulmonary ring to enlarge the right ventricular outflow and the other underwent insertion of a valved aortic homograft conduit. The other five patients ranged in age from 2.5 to 46 years. Although two had partial formation of a pulmonary leaflet, they are included in this series because they had other features of the syndrome including markedly dilated pulmonary arteries. One of these five had, in addition to repair of both the ventricular septal defect and pulmonary infundibular stenosis, excision of anterior portions of the dilated pulmonary arteries and insertion of an aortic homograft valve, one underwent insertion of a right ventricular outflow patch and two underwent enlargement of the pulmonary ring by incision.

FIGURE 6. a, Patient 2. Heart specimen. frontal view shows the narrowed origin of the left pulmonary artery (LPA) arising from ascending aorta (Ao). The aortic arch is left-sided. LAA = left atrial appendage; LAD = left anterior descending artery; LV = left ventricle; PV = pulmonary vein: RPA = right pulmonary artery. b, opened fight ventricle (RV). The aortic valve (AoV) can be seen through the ventricular septal defect (VSD). The conal septum (CS) is deficient and the pulmonary valve leaflets are represented by only a fibrous ridge in the pulmonary ring (Ring). TV = tricuspid valve; other abbreviations as in a. c, Patient 2. Frontal, superior, close-up view of opened infundibulum (lnf), narrowed pulmonary ring (Ring) with absent leaflets and aneurysmally dilated right pulmonary artery (RPA).

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The 46 year old patient, who did not have any interference with the adequate pulmonary ring, died 3.5 years postoperatively. Anomalous Origin of Left Pulmonary Artery From Ascending Aorta Twelve cases of anomalous origin of the left pulmonary artery from the ascending aorta were reviewed.82-g2 The diagnosis was established at postmortem examination in four patients 84,87,89,90who ranged in age from newborn to 9 months. The diagnosis was confirmed at operation in fives2~s3~85~8s~g2 and with angiocardiography in three.s6*g’ Tetralogy of Fallot was present in six patients (50 percent, Table II). The pulmonary valve in the case of Robin et a1.s7 was described as a single flap of endothelium. However, because the right pulmonary artery and pulmonary trunk were hypoplastic, this is not considered a case of absent pulmonary valve leaflets with aneurysmally dilated pulmonary arteries. Tetralogy of Fallot was present in four of the eight patients with a right-sided aortic arch. Successful surgical correction was performed at age 6 weeks to 7 years (average 2.9 years) in five patients with detachment of the left pulmonary artery from the ascending aorta and attachment to the pulmonary trunk.s2,*3,s5,88,g2 Anomalous Origin of the Right Pulmonary Artery From Ascending Aorta Forty-four cases of origin of the right pulmonary artery from the ascending aorta were reviewed.17,84,s6,ss,92-119The diag-

nosis was confirmed by postmortem examination in 28 patients (aged 53 hours to 25 years, median 2 months), at operation in 13 and with angiocardiography in 3. A patent ductus arteriosus was the only associated cardiac anomaly in 25 patients (56 percent); however, three patients were less than 1 month old and the patent ductus may not be a significant lesion in this age group. A further 12 patients had no intracardiac defects. infrequently associated lesions are listed in Table II. The aortic arch was left-sided in the 37 cases in which this information was available. Surgical correction by detaching the right pulmonary artery from the ascending aorta and attaching it to the pulmonary trunk was performed in 15 patients at ages ranging from 7 weeks to 23 years (median 4.5 months and average 3.5 years [3 hospital deaths]). A synthetic conduit, inserted between the right pulmonary artery and pulmonary trunk in eight patients, was positioned anterior to the ascending aorta~,9s.94,s7,105,1~~l14,11s in contrast to the posterior position when the right pulmonary artery was anastomosed directly to the pulmonary trunk.s6,98,‘0’.107,‘08,117Four additional patients died after palliative or incomplete procedures 96,100.115,116 Discussion Absence of the pulmonary valve leaflets is not rare. In 93 percent of the 245 reviewed cases the anomaly was associated with tetralogy of Fallot or a ventricular septal defect. The reported incidence of absent pulmonary valve leaflets in series of cases of tetralogy of Fallot ranges from 2.457 to 6.3S8percent. Although 17 cases of tetralogy of Fallot and absent pulmonary valve leaflets were associated with absence of the origin of the left pulmonary artery, none was associated with absence of the origin of the right pulmonary artery. Anomalous origin of the right pulmonary artery from the ascending aorta was reported more frequently than was that of the left pulmonary artery. However, in the latter instance 50 percent (6 of the 12 cases) were associated with tetralogy of Fallot compared with 4.6 percent (2 of 44 cases) of those with an anomalously arising right pulmonary artery (Table II). In no previous case has the syndrome included absent pulmonary valve leaflets. Diagnosis

FIGURE 7. Patient Postoperative specimen. Opened right ventricular infuncfibulum (Inf), pulmonary ring (Ring) and aneurysmally dilated left pulmonary artery (LPA). The aortic homograft valve conduit has been removed. The right pulmonary artery (RPA) is anastomosed to the pulmonary trunk. No pulmonary valve leaflet tissue is seen in the pulmonary ring. Patch = Teflon patch used to close the ventricular septal defect.

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The diagnosis of the unusual combination of defects (tetralogy of Fallot, absent pulmonary valve leaflets and origin of one pulmonary artery from the ascending aorta) seen in three patients could be suspected clinically. The systolic and diastolic murmurs in a mildly cyanotic patient suggest absent pulmonary valve leaflets wit,h tetralogy of Fall& The aneurysmal dilatation of the hilar pulmonary arteries on the chest roentgenograms would also be compatible with this diagnosis (Fig. 21. Full and jerky pulses point to an aorto-pulmonary communication. The inequality of the size of the hilar pulmonary arteries and the asymmetry of the peripheral pulmonary vasculature with oligemia on the side of the dilated arteries imply separate origins of the pulmonary arteries. Confirmation of the diagnosis requires cineangiocardiography (Fig. 3 and 4). Anatomic Features Right-sided aortic arch: The aortic right-sided in Patient 1 and in 39.4 percent

arch was of the 109

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TABLE I Summary of Surgical Management for Reported Cases of Absent Pulmonary Valve Leaflets Rate

29,49,75 4,7,35,42,50 18,21,77 7,18,42,54,70 4,35,49,54 4,42

Thoracotomy Lobectomy Systemic to PA shunt Pulmonary valvotomy Aneursmorrhaphy of dilated PA Suspension of PA to relieve bronchial compression Reimplantation of PA Closure of VSD Closure of VSD plus Inf resection Cb”,“,;

of VSD. Inf resection and insertion of RVOT

R’epair plus valve insertion ASD closure plus valve insertion

80 100’ 57 50

3 2

14,28,37,44,47,50,72,78 11,81

Five late deaths, four after further surgery. ASD = atrial septal defect; Inf = infundibular; feet.

2

: 5

31,39,74 12,17,35,49,52,53,75,79 3,4,13,18,28,28,31,40,42, 58,84,88,70,72,77 9.53.89.72,79

’

(%I

Patients (n)

References

Procedure

: 40

20 12.5 40

21

24

15 1

13.3 0

l

PA = pulmonary artery; RVOT = right ventricular

reviewed cases with absent pulmonary leaflets in which the side of the arch was stated. The incidence of rightsided aortic arch was 27 percent in our necropsy series of 92 cases with tetralogy of Fallot, excluding the three cases in this report. Absent ductus arteriosus: The ductus arteriosus was absent in our three cases. Emmanouilides et al.Q1 noted a similar finding in their four cases of tetralogy of Fallot with absent pulmonary valve leaflets. This may be coincidental because the status of the ductus in the reviewed cases was seldom stated. In our necropsy study of 64 cases of tetralogy of Fallot and 28 of tetralogy of Fallot with pulmonary atresia, the ductus was absent in 15 and in 10 cases, respectively (that is, 25 of 92 cases, 27 percent). Deficient conal septum: The conal septum in Patient 2 and in the first patient of Layton et a1.37with absent pulmonary valve leaflets was deficient (Fig. 3a and 6b). The resultant type of ventricular septal defect

outflow tract; VSD = ventricular

septal de-

is not the typical defect seen in tetralogy of Fallot because it extends to varying degrees in a more anterosuperior direction, in closer proximity to the pulmonary valve. Nevertheless, if associated with other features of tetralogy of Fallot, the case should be considered to be a variant of tetralogy of Fallot.“* The incidence of this variant was 11.8 percent in our necropsy series of tetralogy of Fallot. Terminology: The origin of a pulmonary artery from the ascending aorta (proximal to the origin of the brachiocephalic arteries) probably differs embryologically from absence of a proximal portion of pulmonary artery with the distal pulmonary artery arising from a ductus. We prefer to call these latter cases absent origin of the right or left pulmonary artery rather than absence of a pulmonary artery, a term that should be reserved for absence of the entire branch. Sotomora and Edwardgo stressed the need for a careful search before excluding the presence of a pulmonary artery. Usually the so-

TABLE II Summary of 56 Reported Cases of Origin of Pulmonary Artery From Ascending Aorta Right Pulmonary Artery

Left Pulmonary Artery

Cases (n) Associated cardiac lesions (n) Associated cardiac defects Tetralogy of Fallot Ventricular septaw defect Pulmonary stenosis Aorticopulmonary window interrupted aortic arch Coarctation of aorta LPA from descending aorta Patent ductus arteriosus Right-sided aortic arch

n

%

References

n

12 5

42

82,83,88,91,92

44 9

6

50

84-87.89.90

1

8

1

8

8

75

% 20

:

4.6 4.6

2 2 ‘1

4.6 4.6 2.3 2.3 56

References 84,92,100,103,104. 111,115 108,112 101,110

91

83

2:

102,105 106 84 96 17,84,86,89,90,92-95, 97-99,104,107,109, 113,119

82-84,88-92

LPA = left pulmonary artery.

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called “absent” right or left pulmonary artery is actually present and supplied either by the ductus arteriosus or by collateral vessels.

elude plication or excision of portions of the dilated pulmonary arteries, if these are producing bronchial compression, in addition to repair of the associated defects.

Management

Controversy exists regarding the need to insert a valve in the pulmonary ring in cases with absent pulmonary valve leaflets. Pinsky et a1.53do not recommend

The pathologic problems in our three patients are potentially surgically correctable: The tetralogy type

of ventricular septal defect can be closed with a prosthetic patch; infundibular stenosis, if present, can be excised; the narrowed pulmonary ring can be enlarged by insertion of a pericardial patch; and the anomalously arising pulmonary artery can be anastomosed to the pulmonary trunk. The dilemma in management of patients with absent pulmonary valve leaflets occurs in infants with respiratory problems related to bronchial compression.35 The surgical mortality rate is formidable (66

percent), but the median age at death in nonsurgically treated patients is 3 months. Pinsky et a1.53stated that repair in infancy does not relieve the respiratory symptoms and advocate vigorous continued respiratory therapy for infants and small children. However, two of their three infants managed in this manner required a total of 21 hospital admissions before the age of 16 months. It is possible that surgical treatment could in-

it; Stafford et a1.72are concerned about the long-term complications of valve insertion and they conclude that further experience is needed to determine the exact role of a valved conduit in the treatment of these cases; and Ilbawi et a1.28and Layton et a1.“7recommend valve insertion. At present we favor the insertion of a homograft aortic valve conduit in young patients, particularly in those with elevated pulmonary arterial pressure and in those with cardiomegaly and congestive heart failure secondary to increased pulmonary blood flow. The provision of a competent outflow valve in the right ventricle may improve the function of this ventricle and hence increase hospital survival. Addendum Since this manuscript was prepared, we have become aware four additional cases of absent pulmonary valve leaflets and tricuspid atresia.123J24 of

References 1. Anselmi G, Munor AS, Esplno Vela J, Perez-Sot0 J, Villegas M, Yonroy G. Agenesia de las valvulas sigmoideas pulmonares: presentacio de dos case y revision de la literatura. Arch lnst Cardiol Mex 1960; 30:409-27. 2. Baker WP, Kelminson LL, Turner W Jr, Blount SG Jr. Absence of pulmonic valve associated with double-outlet right ventricle. Circulation 1977; 36:452-5. 3. Borg SA, Young LW, Roghaer GD. Congenital avalvular pulmonary artery and infantile lobar emphysema. A diagnostic correlation. Am J Roentgen01 1975; 125412-21. 4. Bove EL, Shaher RM, Alley R, McKneally M. Tetralogy of Fallot with absent pulmonary valve and aneurysm of the pulmonary artery: report of two cases presenting as obstructive lung disease. J Pediat 1972;81:339-43. 5. Cameron AH. Malformations of thymus and cardiovascular system (abstr). Arch Dis Child 1965;40:334. 6 Campeau L, Ruble PE, Cooksey WE. Congenital absence of the pulmonary valve. Circulation 1957;15:397-404. 7. Campeau L, Gilbert G, Aerichide N. Absence of the pulmonary valve. Report of two cases associated with other congenital lesions Am J Cardiol 1961;8:113-24. 8. Carrera A, Ascuna JI, g’Arate F, lrarte M. Agenesia de la valvula pulmonar con tetralogia de Fallot. Rev Esp Cardiol 1971;24: 259-65. 9. Castaneda AR, Freed MD, Williams RG, Norwood WI. Repair of tetralogy of Fallot in infancy. Early and late results. J Thorac Cardiovasc Surg 1977;74:372-81. 10. Chevers N. A collection of facts illustrative of the morbid conditions of the pulmonary artery, as bearing upon the treatment of cardiac and pulmonary diseases, Lond Med Gaz 1846;38: 828-35. 11. Chlemmong Koltlp P, Replogle RL, Gonzalez-Lavin L, Arcilla RA. Congenital absence of the pulmonary valve with atrial septal defect surgically corrected with aortic homograft. Chest 1972; 62:100-3. 12. Chllders RW, McCrea PC. Absence of the pulmonary valve. A case occurring in the Marfan’s syndrome. Circulation 1964;29: 598-603. 13. Cohen LS, Roberts WC. Tetralogy of Fallot. Its unusual variants and it simulators. Chest 1970;57:266-74.

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