Echocardiographic identification of aortic atresia with ventricular septal defect, normal left ventricle and mitral valve

Volume Number

Table

113 6

Brief

Communications

1521

I. Clinical, electrocardiographic, and morphologic features

Case no.

Origin

Age (Yr)

& sex

Chest pain

Murmur

Chest x-ray

Resting ECG

Exercise ECG

Thallium scintigr.

CHD

Treatment Beta blocker Beta blocker Op.

45

op. Beta blocker Beta blocker Beta blocker

15 12

1

55F

+

C

Normal

Normal

Normal

Normal

LAD

0

2

62M

+

C

Normal

Normal

Normal

Normal

LAD

0

3

56M

+

C

Normal

Normal

Normal

60M 52F

+ +

C C

Normal Normal

ST1 Normal

(-) Normal

LAD RCA LAD LM

0

4 5

RBBB LAH IMI Normal

6

53M

+

S

Normal

Normal

Normal

Normal

LM

0

I

32M

+

S

Normal

Normal

Normal

Normal

LM

0

Abbreviations axis deviation; S = systolic;

and symbols: C = continous; F = female; IMI = inferior myocardial LM = left main coronary artery; M = male; RBBB = right bundle 0 = absent; f-1 = not done; + = present.

sisin the absenceof surgery, the possibility of recurrences of coronary artery fistula after surgical repair, and the high rate of surgical complications are additional reasons for avoiding operations in asymptomatic or mildly symptomatic patients with small shunts.3*6We, for example, were not able to demonstrate seriousfistula-related complications in any of our patients in spite of an averageage of 53 years at the time of diagnosis. Our results concur with those of previous studies,3s5 which report only single casesof endocarditis, embolism,fistula rupture, or pulmonary hypertension and rare instances of myocardial infarction. On the other hand, Liberthson et a1.,3who reviewed the literature concerning 119 surgically treated patients, found 23% perioperative complications including 7% mortality and 7% myocardial infarctions. Moreover, angina pectoris, which is often consideredan indication for surgery, although an objective proof of ischemiais rarely possible, seems to respond quite well to beta blockers. This is shown by the pronounced improvement of symptoms in five of our six primarily medically treated patients. However, in none of these cases was there objective evidence (exercise ECG, thallium scintigraphy) for ischemia.

REFERENCES

1. Alpert BS, Neal MJ, Stewart GS. Coronary arteriovenous fistula-a local epidemic. Am J Cardiol 1984;54:683. 2. Gobel FL, Anderson CF, Baltaxe HA, Amplatz K, Wang Y. Shunts between the coronary and pulmonary arteries with normal origin of the coronary arteries. Am J Cardiol 1970; 25:655: 3. Liberthson RR, Sagar K, Berkoben JP, Weintraub RM, Levine FH. Congenital coronary arteriovenous fistula. Circulation 1979;59:849. 4. Macri R, Capulzini A, Faxxini L, Cornali M, Verunelli F, Reginato E. Congenital coronary artery fistula: Report of five patients, diagnostic problems and principles of management. Thorac Cardiovasc Surg 1982;30:167. 5. Lowe JE, Oldham HN, Sabiston DC. Surgical management of congenital coronary artery fistulas. Ann Surg 1981;194:373.

infarction: branch block;

Follow-up fmos)

of

fistula

+ 0

LAD = left anterior descending RCA = right coronary artery;

6. Friedman DM, Rutkowski pulsed Doppler/two-dimensional Am J Cardiol 1985;55:1652.

M.

31 24

10 6

coronary artery; ST1 = ST segment

Coronary artery echocardiographic

LAH = left depression;

fistula: A study.

Echocardiographic identification of aortic atresia with ventricular septal defect, normal left ventricle and mitral valve Bruno Marino, M.D., Stephen P. Sanders,M.D., Ira A. Parness,M.D., and Steven D. Colan, M.D. Boston, Mass., and Rome, Italy

With the advent of palliative and physiologically corrective operations for patients with aortic atresia and hypoplastic left heart syndrome,’ several investigator8’ have outlined the importance of associatedanatomic abnormalities such as coarctation of the aorta or dysplastic tricuspid valve. Recent evidence5indicates that two-dimensional’ echocardiography (2-D echo) can provide all of the anatomic information necessaryto perform palliative surgery in patients with hypoplastic left heart syndrome. Patients with a normal left ventricle and mitral valve in associationwith a large ventricular septal defect form an important although uncommon subset of patients with aortic atresia.6-8Palliative surgery in such cases6~g~10 is usually

From the Department of Pediatric Rome.

quite

different

Department of Pediatrics, Cardiology

Reprint requests: Stephen The Children’s Hospital,

from

the procedure

for hypoplastic

of Cardiology, The Children’s Hospital and the Harvard Medical School; and The Department and Cardiac Surgery, Bambino Gesu Hospital, P. Sanders, 300 Longwood

M.D., Ave.,

Department Boston, MA

of Cardiology, 02115.

1522

Brief Communications

American

June 1987 Heart Journal

Fig. 1 A and 6. A, Parasternal long-axis view in a patient with aortic at&&m and a normal left ventricle. The hypoplastic ascendingaorta can be seenarising above,the left ventricle. B, Subxiphoid short-axis view illustrating a conoventricular ventricular septal defect.

Fig. 1 C. Suprasternal notch views showing a discrete juxtaductal coarctation (arrow) in case No. 2. Note the hypoplastic ascending aorta. Abbreviations: A = aorta; Asc Ao = ascending aorta; Dsc = descending aorta; LA = left atrium, LSCA = left subclavian artery; LV = left ventricle; P = puhnonary artery; RV = right ventricle.

left heart syndrome,‘~* sincea biventricular repair may be possible.Therefore, we reviewed our experience.with 2-D echo in four patients with aortic atresia and a normal left ventricle to evaluate the’ reliability of 2D echo for establishing thii diagnosisand for detecting associatedlesions.

Review of the files of the Noninvasive Laboratories at The Children’s Hospital, Boaton, Massachusetts, and The Bambino Gesu Hospital, Rome, Italy, ‘yielded four patients, ages 2 to 21 days, with aortic atresia and a normal left ventricle in association with a ventricular septal defect (Table I). All four patients presented in the neonatal period with congestiveheart failure and/or circulatory collapse. The 2-D echowasperformed with a Picker Hchoview 80 CT (Picker International, Inc., Northford, Conn.) or an ATL Mark 500 or 600 (Advanced’ Technology Laboratories, Inc., Bellevue, Wash.), equipped with a 5.0 MHz transducer. In three casesa complete examination was performed, including subxiphoid, apical, parastemal, and suprasternal notch views. In one patient only subxiphoid view were obtained. Left ventricular end-diastolic volume was calculated with a Franklin Quantic 1200 Review Center (Bruce Franklin, Inc., Seattle, Wash.) from subxiphoid long-axis views of the left ventricle by meansof a % area-length algorithm where: volume = 3% x cross-sectional area in short axis X long axis length. Aortic root diameter was measured in the pamsternal long-axis view just above the sinusesof Valsalva. The mitral anulus diameter was measuredin early d&stole by use of apical or subxiphoid four-chamber views. Both measurements were adjusted for the cubic root of body surface area. The diagnosisof aortic atresia with normal leftventricle

Volume Number

113 6

Table

Brief

Communications

1523

I. Anatomic and volume data for the four patients with aortic atresia and normal left ventricle BSA

Case

Age (days)

1

2

0.24

Conoventricular

2

11

0.22

Perimembranous

3

2

0.17

Conoventricular

4

22

0.22

Perimembranous

Abbreviations: PDA = patent

Cm?

VSD

BSA = body surface area; ‘m ductus arteriosus; VSD = ventricular

Aortic

arch

Interruption type C Juxtaductal coarctation Interruption type C Normal

= cubic root of body septal defect; AX

PDA

LVEDV (mllm3

ASC A0 diameter (cm/’ vG!ESij

Bilateral

32.1

1.6

Left

60.0

1.6

0.5

Left

35.3

1.3

0.5

Left

50.0

1.5

0.3

surface area; LVEDV = left ventricular A0 = ascending aorta.

and mitral valve was established prospectively by 2-D echo in all four cases.Parasternal long-axis views (Fig. 1, A) were best for demonstrating the atretic aortic valve and hypoplastic ascendingaorta, although thesefeatures could also be seenin subxiphoid views. The type of ventricular septal defect, conoventricular in two patients and perimembranous in two others, was correctly diagnosed by 2-D echo. The ventricular septal defect wasbest seenwith the subxiphoid short-axis views (Fig. 1, B). The aortic arch anatomy was not investigated echocardiographically in the first patient in our series.Interruption of the aortic arch (type C) was subsequently demonstrated at cardiac catheterization. In the other three patients the aortic arch anatomy was correctly delineated by 2-D echo. In one patient the aortic arch was normal. In another, a discrete juxtaductal coarctation was demonstrated (Fig. 1, C). Interruption of the aortic arch between the carotid arteries (type C) with aberrant right subclavian artery was demonstrated in the third patient. A large patent ductus arteriosus was present in all four patients. The left ventricular end-diastolic volume index for these four patients (Table I) ranged from 32.1 to 60.0 ml/m2 (normal for our laboratory = 40 + 10 ml/m*). The mitral valve diameter was 1.3 to 1.6 cm/3V’i%& and the ascending aorta diameter was 0.3 to 0.5 cm/3m. Because of recent successwith palliative surgery’ for patients with aortic atresia, precise anatomic diagnosisis now mandatory. Caseswith a large ventricular septal defect, normal left ventricle, and mitral valve represent a rare subgroup of patients with aortic atresia and are potential candidates for biventricular repair.6~g~10 The palliative surgery for thesepatients differs from that used in classicalaortic atresia with hypoplastic left ventricle.’ Thus, the distinction between these two entities must be made accurately. This report illustrates that 2-D echo is capable of identifying those patients with aortic atresia and a normal left ventricle as well as delineating the type of ventricular septal defect (VSD) and the anatomy of the aortic arch. The diagnosis of aortic atresia, instead of critical aortic stenosis, was based on the absence of systolic motion of the valve leaflets and the extreme hypoplasia of the aortic root. In the more recent cases, interrogation of the ascendingaorta with Doppler indi-

MV diameter (cm/-’ dB=)

end-diastolic

volume;

MV

= mitral

valve;

cated no anterogradesystolic flow. Of interest, both of our caseswith interruption of the aortic arch had associated conoventricular septal defects with posterior deviation of the infundibular septum, as has been reported in other casesof interruption of the aortic arch.” This combination of aortic atresia, posterior malalignment VSD, and normal left ventricle and mitral valve appears to be a syndrome that varies predominantly in the aortic arch anatomy. As with other varieties of aortic atresia, the anatomic findings can be well defined by 2-D echo. REFERENCES

1. Norwood WI, Lang P, Hansen D. Physiologic repair of aortic atresia-hypoplastic left heart syndrome. N Engl J Med 1983;308:23. 2. Bharati S, Lev M. The surgical anatomy of hypoplasia of aortic tract complex. J Thorac Cardiovasc Surg 1984;88:97. 3. Hawkins JA, Doty DB. Aortic atresia: Morphologic characteristics affecting survival and operative palliation. J Thorac Cardiovasc Surg 1984;88:620. 4. Jonas RA, Lang P, Hansen D, Hickey P, Castaneda AR. First stage palliation of hypoplastic left heart syndrome: The importance of coarctation and shunt size. J Thorac Cardiovast Surg 1986;92:6-13. 5. Bash SE, Huhta JC, Gutgesell HP, Ott DA. Echocardiography: Is it accurate enough to guide surgical palliation of hypoplastic left heart syndrome? J Am Co11 Cardiol 1986; 7:610. 6. Freedom RM, Williams WG, Dische MR, Rowe RD. Anatomical variants in aortic atresia. Potential candidates for ventriculoaortic reconstitution. Br Heart J 1976;38:821. 7. Marino B, Thiene G, Bosman C, Milanesi 0, Gallo P, Pellegrino PA. Profilo anatomo-clinic0 dell’atresia aortica con difetto interventricolare. Rev Latina Cardiol 1980; 1:108. 8. Thiene G, Gallucci V, MaCartney FJ, Del Torso S, Pellegrino PA, Anderson RH. Anatomy of aortic atresia. Cases presenting with a ventricular septal defect. Circulation 1979; 59;173. 9. Norwood WI, Stellin GJ. Aortic atresia with interrupted aortic arch. Reparative operation. J Thorac Cardiovasc Surg 1981;81:239. 10. Duffy CE, Muster AJ, DeLeon SY, Idriss FS, Ilbawi M, Riggs TW, Paul MH. Successful surgical repair of aortic atresia associated with normal left ventricle. J Am Co11 Cardiol 1983;1:1503. 11. VanPraagh R, Bernhard WF, Rosenthal A, Parisi LF, Flyer DC. Interrupted aortic arch. Surgical treatment. Am J Cardiol 1971;27:200.