Isolated innominate artery in asplenia syndrome with aortic atresia: Newly recognized cardiovascular complex

May 1996

1042 Papagiannis et al.

American Heart Journal

Isolated innominate artery in asplenia syndrome with aortic atresia: Newly recognized cardiovascular complex

were single, a n d a grade III/VI holosystolic m u r m u r was h e a r d along the right u p p e r s t e r n a l border. The liver was palpable at the left costal m a r g i n and the blood pressures were noteworthy: right a r m 74/44 m m Hg, left a r m 31/21 m m Hg, right leg 63/37 m m Hg, and left leg 57/40 m m Hg. Chest films showed dextrocardia with a right aortic arch and a symmetric liver. Two-dimensional echocardiography, cardiac catheterization, a n d angiocardiography (Figs. 1 a n d 2) revealed complex congenital h e a r t disease. A t surgery, because the p a t i e n t was thought to have truncus arteriosus, the p u l m o n a r y a r t e r y branches were detached from the m a i n t r u n k a n d were joined in the midline. Because the p a t i e n t h a d a large morphologically right ventricle (left sided) and a small morphologically left ventricle (right-sided), the p u l m o n a r y circulation was supplied by a 3.5 m m polytetrafluoroethylene (Gore-Tex, Gore a n d Associates, Newark, Del,) central s h u n t from the m a i n t r u n k to the left p u l m o n a r y artery. The isolated left innominate artery, which rose from the left p u l m o n a r y a r t e r y v i a a left p a t e n t ductus arteriosus, was r e i m p l a n t e d into the m a i n trunk. The infant could not be weaned from cardiopulmon a r y bypass and died in the operating room. Autopsy revealed asplenia syndrome with visceral situs ambiguous (A); dextrocardia (Fig. 3); double-outlet right ventricle (DORV) with a t r i a l situs inversus (I), concordant ventricular L-loop (L), and D-malposition of the g r e a t arteries (D), t h a t is, DORV {A(I), L, D}; common atrium; n o r m a l systemic and p u l m o n a r y venous connections for a t r i a l inver-

John Papagiannis, MD, a Ronald J. Kanter, MD, b Richard S. V a n d e r Heide, MD, c Keith A. Reimer, MD, ¢ Ross M. Ungerleider, MD, d and Richard Van Praagh, MD a Boston, Mass., and Durham, N. C. A previously u n r e p o r t e d combination of cardiovascular anomalies is presented with isolation of the innominate a r t e r y (that does not rise from the aortic arch) 16 in association with the asplenia syndrome a n d aortic atresia. A 3.48 kg black boy was referred at age 1 day to Duke University Medical Center for a s s e s s m e n t ofdextrocardia. Physical examination revealed t h a t the cardiac impulse was m a x i m a l in the right t h i r d intercostal space, $1 and $2

From the aDepartments of Pathology and Cardiology, Children's Hospital, Boston; and the Departments ofbpediatrics, Cpathology, and dCardiac Surgery, Duke University Medical Center, Durham. Reprint requests: Richard Van Praagh, MD, Children's Hospital, Cardiac Registry, 300 Longwood Ave., Boston, MA 02115. AM HEARTJ 1996;131:1042-4. Copyright © 1996 by Mosby-Year Book, Inc. 0002-8703/96/$5.00 + 0 414/70094

Table I. Findings in cases of isolated innominate a r t e r y Age

Sex

Aortic arch

Martin et al. 1

8.5 yr

F

R

PDA (L)

Reduced L arm BP

Garti and Aygen2

49 yr

F

R

None

Park et al. 8 Crumpet al.4

3 days 2 days

M F

R R

CCAVC, PDA (R) CCAVC, bilateral SVC, polysplenia

Fong and Venables5

6 wk

F

R

Pantke et al. 6

1 day

M

L

Present case

1 day

M

R

CHARGE association, bilateral PDA AoAt, polysplenia, multiple congenital anomalies Dextrocardia, DORV, CCAVC, AoAt, HLV, bilateral PDA, asplenia

Syncope, L arm weakness CHF CHF, seizures, reduced L arm BP CHF

Author

Associated anomalies

Symptoms and signs

Not reported

Reduced L arm BP

Outcome

Successful connection LInnA to aortic arch Not reported Death (from CHF) Death (intraoperative) Death (sudden) Death (intraoperative, during repair of encephalocele) Death (intraoperative, during attempted Stage I palliative procedure and LInnA reimplantation)

BP, Bloodpressure; AoAt, aortic atresia; CCAVC,completecommonatrioventricular canal; CHF,congestiveheart failure; DORV, double-outletright ventricle; HLV, hypoplasticleft ventricle;L, left; LInnA, left innominate artery; PDA,patent ductus arteriosus; R, right; SVC, superior vena cava; sz, seizure.

Volume 131, Number 5

Papagiannis et al.

American Heart Journal

RSA4 ~

LCCA.

1043

LSA

RAoA__ ~ ~-"/RP?A , ~ L l n n A

Fig. 2. Tracing of Fig. 1. AAo, Ascending aorta. Other abbreviations as in Fig. 1.

Fig. 1.

Posteroanterior projection of selective injection into main pulmonary artery (MPA), giving rise to right pulmonary (RPA) and left pulmonary (LPA) branches. MPA communicates with right descending thoracic aorta (DAo) via right patent ductus arteriosus (RPDA), right DAo veering leftward at level of diaphragm. Left innominate artery (LInnA) arises from LPA via left patent ductus arteriosus (LPDA) and gives origin to left common carotid artery (LCCA) and to left subclavian artery (LSA). Hypoplastic ascending aorta above atretic aortic valve is confluent with right aortic arch (RAoA), which gives origin to right common carotid artery (RCCA) and right subclavian artery (RSA). Anomalous origin of LInnA from LPA creates erroneous impression of type C aortic arch interruption (between RCCA and LCCA).

sion, with inverted atrial appendages (large, triangular, anterior appendage of the morphologically right atrium, left-sided, and small finger-shaped, posterior appendage of the morphologically left atrium, right-sided); complete common atrioventricular canal with common atrioventricular valve connecting only with the morphologically right ventricle, left-sided and anterior; hypoplastic morphologically left ventricle, right-sided and posterior; aortic atresia; right aortic arch (Figs. 1 and 2); isolated left innominate artery that preoperatively had risen from the left pulmonary artery via a left patent ductus arteriosus; and bilateral patent ductus arteriosi (Fig. 2). Developmentally, congenital isolation of the left innominate artery is believed to result from two interruptions of the aortic arch system (Fig. 4): (1) interruption of the left limb of the aortic sac proximal to the left common carotid artery (upper arrow), and (2) interruption of the left dorsal aorta distal to the seventh intersegmental artery (low-

Fig. 3.

Postmortem specimen of heart, lungs, and great arteries, seen from the front and above. Central shunt between MPA and confluence of RPA and LPA is seen. Isolated LInnA has been anastomosed to MPA. Note large morphologically right atrial appendage (RAA; left-sided), smaller morphologically left atrial appendage (right-sided), showing absence of right atrial appendage isomerism in heterotaxy syndrome with asp]enia, and characteristically large morphologically right ventricle (RV), left-sided, and much smaller morphologically left ventricle (LV), right-sided. Other abbreviations as in Fig. 1.

er arrow). The unique finding in this case of asplenia with aortic atresia is the coexistence of isolation of the left innominate artery; this is one of the rarest forms of congenital cardiovascular disease, with only six cases having been reported previously to our knowledge (Table I). 1-6DORV {I, L, D} is an exceedingly rare form of double-outlet right

May 1996

1044

Culclasure, Dorogy, and Enzenauer

American Heart Journal

Cryoglobulinemia: A reversible cause of dilated cardiomyopathy Talley F. Culclasure, MD, Mark E. Dorogy, MD, and Raymond J. Enzenauer, MD Aurora, Colo.

Asc

LPA MPA

,

LSA

Fig. 4. Diagram of aortic arch system of 14 mm human embryo, seen from front, estimated age 36 to 38 days (after Congdon7). Upper arrow (left horn of aortic sac) and lower arrow (distal end of left dorsal aorta) indicate sites of interruption of aortic arch system that result in isolated LInnA. Lower interruption produces RAoA. Upper interruption separates LInnA from RAoA, forcing LInnA to rise from LPA via LPDA. Asc Ao, Ascending aorta. Other abbreviations as in Fig. 1.

ventricle. The only other patient we have ever seen with this segmental anatomy (A63-94) did have the asplenia syndrome but did not have aortic valvar atresia or isolation of the innominate artery. REFERENCES

1. Martin EC, Mesko ZG, Hailer JO, Gordon DH. Isolation of the left innominate artery, right arch and a left patent ductus arteriosus. Am J Roehtgenol 1979;132:833-5. 2. Garti IJ, Aygen MM. Right aortic arch and isolation of left innominate artery from the aorta. Cardiovasc Intervent Radiol 1982;5:235-7. 3. Park MK. Right aortic arch with isolation of the left innominate artery. Chest 1979;76:106-8. 4. Crump WD, Dische MR, Anthony CL. Right aortic arch, isolated left common carotid and left subclavian arteries and subclavian steal syndrome: a variant of polysplenia syndrome. Hum Pathol 1981;12:. 936-8. 5. Fong LV, Venables AW. Isolation of the left common carotid or left innominate artery. Br Heart J 1987;57:552-4. 6. Pantke OA, Gorlin RJ, Burke BA. Polysplenia syndrome with skeletal and central nervous system anomalies. Birth Defects 1975; 11:252-63. 7. Congdon ED. Transformation of the aortic-arch system during the development of the human embryo. Contrib Embryol Can]egie Inst of Washington 1922;14:47-110.

Cryoglobulinemia is defined as the presence of immunoglobulin molecules that reversibly precipitate at low temperatures. In essential mixed cryoglobulinemia (EMC), circulating immune complex deposition may cause a small vessel vasculitis most commonly involving the skin, kidneys, and liver. 1 Cardiac involvement in this disorder is rare, but up to 21% of deaths have been attributed to acute infarction or refractory heart failure. 2, 3 The development of heart disease has been related to coexisting valvular or presumed atherosclerotic heart disease. 2, 3 We describe a patient with EMC having symptoms of heart failure and severe left ventricular (LV) dysfunction who experienced a rapid recovery of LV function after plasmapheresis. A 65-year-old man With a 5-year history of EMC was seen after having several days of progressive dyspnea, chest discomfort, paroxysmal nocturnal dyspnea, and orthopnea. He was previously treated with multiple courses of corticosteroids, immunosuppressive agents, and plasmapheresis but had recently declined further therapy. His past medical history was remarkable for chronic renal insufficiency (creatinine 2.0 mg/dL) from cryoglobulinemia. He appeared tachypneic and tachycardic on admission. Marked jugular venous distension, bilateral pulmonary rales, a third heart sound, and palpable lower extremity purpura were noted, but there was no peripheral edema. The blood urea nitrogen and creatinine were elevated (61 mg/dl and 6.0 mg/dl, respectively), consistent with acute renal failure. A room temperature cryocrit was 8% (normal, negative), and cardiac enzymes were normal. His initial chest radiograph demonstrated pulmonary edema, and the electrocardiogram revealed a sinus tachycardia with a left atrial abnormality and left axis deviation. The patient was admitted to the intensive care unit for invasive hemodynamic monitoring. Initial measurements were notable for a central venous pressure of 20 mm Hg, a pulmonary artery occlusion pressure of 25 mm Hg and a cardiac index of 1.5 L/min/m 2. Transthoracic echocardiography (TTE) demonstrated severe LV dysfunction with an LV ejection fraction calculated by the modified Simpson's

From the Cardiology Service and Rheumatology Service, Fitzsimons Army Medical Center. The opinions or assertions contained herein are those of the authors and are not to be construed as official policy of the Department of the Army or the Department of Defense. Reprint requests: Tailey F. Culclasure, MD, Cardiology Service, Department of Medicine, Fitzsimons Army Medical Center, Aurora, CO 800455001. AM HEARTJ 1996;131:1044-6. 4/4/70760