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Aortic arch thrombosis in the neonate Miguel Sousa Uva, Alain Serraf, François Lacour-Gayet, Jacqueline Bruniaux, Daniel Sidi, Jean Kachaner, Jean-Louis Cloez, Laurent Ferment and Claude Planché Ann Thorac Surg 1993;55:990-992 DOI: 10.1016/0003-4975(93)90132-2

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The Annals of Thoracic Surgery is the official journal of The Society of Thoracic Surgeons and the Southern Thoracic Surgical Association. Copyright © 1993 by The Society of Thoracic Surgeons. Print ISSN: 0003-4975; eISSN: 1552-6259.

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CASE REPORTS

Aortic Arch Thrombosis in the Neonate

Miguel Sousa Uva, MD, Alain Serraf, MD, Francois Lacour-Gayet, MD, Jacqueline Bruniaux, MD, Daniel Sidi, MD, Jean Kachaner, Jean-Louis Cloez, MD, Laurent Fermont, MD, and Claude Planche, MD

Department of Pediatric Cardiac Surgery, Centre Chirurgical Mane Lannelongue, Le Plessis Robinson, France

Two cases of neonatal aortic arch thrombosis are reported. One patient, who had ascending aortic thrombosis, died preoperatively. The other had reoperation and is alive and well at 6 months' follow-up. No obvious cause w s found. a (Ann Thoruc Surg 1993;55:990-2)

ortic arch thrombosis of the neonate is a rare and highly lethal event. Five cases were reported in the literature, with 2 survivors [ld]. We report 2 cases of neonatal thrombosis of the aortic arch, 1 of which was successfully operated on, and discuss the literature concerning this rare entity.

A

Case Reports Patient 2

A male infant weighing 3,340 g was born to a 31-year-old woman at 40 weeks' gestation. Prenatal history revealed a 220 V domestic electrocution at 37 weeks' gestation. On the day of delivery fetal bradycardia prompted an emergent cesarean section. Apgar score was 8 at 5 minutes. Five hours after birth diffuse cyanosis refractory to oxygen developed. Respiratory rate was 62/min, and peripheral pulses were faint; arterial pressure was 48/30 mm Hg in the left arm, 42/27 mm Hg in the leg, and 72/45 mm Hg in the right arm. No murmur was present. A 2-cm liver edge was palpated. Chest radiography demonstrated an enlarged heart with normal pulmonary vascular pattern. Electrocardiography showed a sinus rhythm at 180/minwith negative T waves in the precordial leads. Two-dimensional echocardiography was performed upon arrival at the referring hospital and demonstrated a dilated left ventricle with a shortening fraction of 0.15 (normal range, 0.30 to 0.45) and an enlarged ascending aorta, progressively narrowing at its junction with the transverse aorta. This was interpreted as an interrupted aortic arch. Mechanical ventilation, correction of acidosis, and prostaglandin El (0.1 pg kg-' * min-`) infusion were then started. Cardiac catheterization was performed 18 hours after birth and showed the following pressures: left ventricle, 90/12 mm Hg (oxygen saturation = 95%),

pulmonary artery, 4515 mm Hg, descending aorta, 45/25 nun Hg (oxygen saturation = 40%). Angiography showed a hypokinetic left ventricle and obstruction of the ascending aorta at the level of the right brachiocephalic artery (Fig 1); we concluded that there was an occlusion o f the ascending aorta by an intraluminal obstacle at the level of the right brachiocephalic artery. Upon arrival at the operating room, 22 hours after birth, hemodynamics had further deteriorated (systolic arterial pressure, 40 mm Hg; pH, 7.27), and induction of anesthesia provoked extreme bradycardia and a nonpulsed arterial pressure followed by ventricular fibrillation. Autopsy was performed and revealed organized thrombus above the aortic valve up to the origin of the left subclavian artery. No coactation was seen. Aortic diameters were as follows: ascending, 6.3 mm; transverse, 4.4 mm; and descending, 5.7 mm. A large patent ductus was present. The left ventricle was markedly dilated and hypertrophied (left ventricular wall thickness, 9 mm). No structural anomalies of the mitral valve or the left ventricular outflow tract were noted. Histologic examination of the thrombus was not performed.

Patient 2

A 34-year-old woman gave birth at term to a male child weighing 2,170 g. Pregnancy had been normal besides a urinary tract infection and a premature rupture of the amniotic sac. Apgar score was 9 at 10 minutes. On the second day a coarctation syndrome was diagnosed because of heart failure, absent femoral pulses, and hypertension in the right arm. Two-dimensional echocardiography showed an intraaortic dense image in the transverse arch. The baby underwent a left posterior thoracotomy. Externally and after incision the aorta had a normal aspect. Although no coarctation was present the aortic isthmus was resected. A white and adherent thrombus was removed from the transverse aorta just distal to the right brachiocephalic artery, and the aorta was reconstructed by an end-to-end anastomosis. Pathologic examination revealed a fibroblastic endarteritis of the aortic wall and an organized partially calcified thrombus. Postoperatively, two-dimensional echocardiography showed a residual thrombus in the aortic arch (Fig 2). Heparin administration was started, but a right arm and leg neurologic deficit occurred, corresponding to an ischemic lesion on the cerebral computed tomographic scan. Aortography and hemodynamics further confirmed rethrombosis and narrowing with a 50-mm Hg gradient across the aortic arch (Fig 3). The patient was reoperated

0003-4975/93/$6.00

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Accepted for publication May 26, 1992. Address reprint requests to D Plan&&, Department of Pediatric Cardiac r Surgery, Mane Lannelongue Hospital, 133, ave de la Resistance, 92350 Le Plessis Robinson, France. 0 1993 by The Society of Thoracic Surgeons

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Ann Thorac Surg 1993;55:990-2

CASE REPORT UVA ET AL AORTIC THROMBOSIS IN THE NEONATE

991

Fig 2 . (Patient 2.) Postoperative two-dimensional echocardiography of

the aortic arch shows residual narrowing of the transverse aorta and isthmus after simple aortic thrombectomy (arrow). Pulsed Doppler shows a 50-mm Hg gradient across the isthmus. (a = aortic arch; d = descending aorta.)

Fig 1 . (Patient 1 .) Angiography shows crescent-shaped obstruction of

the ascending aorta and marked left ventricular dilatation.

on through the same route. White fibrinous debris was found adherent to a thickened aortic wall. The aorta was closed with a Gore-Tex* patch, extending over the origins of left carotid and left subclavian arteries. Postoperatively the patient did well; femoral pulses were normal. No cause was found; namely, levels of antithrombin 111, protein S, and protein C were normal. The infant has been followed up for 6 months. Blood pressure and development are normal. Two-dimensional Doppler echocardiography shows a free and normal-sized aorta with no gradient.

eration should not be delayed as occurred in our first patient, who died at induction of anesthesia. Some authors have suggested a trial of fibrinolytic agents before operation [6]. Although we have no experience with this treatment modality, we, like others [7], consider these to

Comment

Thrombosis of the aortic arch in the neonate is extremely rare, and its cause is unknown. It carries a high mortality, as most of the reported cases are postmortem [4]. Only 5 cases of "spontaneous" neonatal aortic arch thrombosis were reported in the literature. Two of the patients died before operation [4, 51. Three neonates were operated on with 2 survivors. Table 1 summarize these cases. Clinical presentation is that of a newborn less than 3 days old in critical condition. As in interrupted aortic arch, cyanosis of the lower body, heart failure, and unequal or absent pulses are the most frequently encountered manifestations. Diagnosis is confirmed by twodimensional echocardiography and cardiac catheterization. Continuous prostaglandin infusion to maintain systemic circulation and correction of acidosis are necessary to obtain hemodynamic stabilization. However, op-

Gore-Tex vascular graft, registered trademark o W.L. Gore and Assof ciates, Inc, Elkton, MD.

Fig 3. (Patient 2 . ) Aortography performed after thrombectomy and primary aortic closure shows residual stenosis and rethrombosis.

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992

CASEREPORT UVAETAL N AORTIC THROMBOSIS I THE NEONATE

Ann Thorac Surg

1993:55:99&2

Table 1 . Aortic Arch Thrombosis in the Neonate: Reported Cases in the Literature

First Author McFaul 111 Hamilton [2] Conti (31 Trowitzsch [4] Scott (51 Age (days)

1 1 1

Weight (kg)

2.8 2.8 2.6 4.3 2.9

Surgical Treatment MS, DHCA, thrombectomy LT, thrombectomy, patch closure LT, thrombectomy, endarterectomy, patch closure No operation Thrombectomy, patch closure

~

Thrombus Aspect and Location

Outcome Died day 2 Favorable Favorable Died Died day 1

LV = left

No histology; ascending + transverse

aorta Organized, distal to LCA

Organized, distal to RBCA Organized, ascending aorta to LSCA + LV Organized, ascending + transverse aorta

LT = lateral thoracotomy;

1

2

LSCA = left subclavian artery; LCA = l f carotid artery; et DHCA = deep hypothermic circulatory arrest; ventricle; MS = median stemotomy; RBCA = right brachiocephalic artery.

be critically ill newborns with organized thrombi and therefore see no indication for thrombolysis in this setting. Left thoracotomy and longitudinal aortotomy over o the transverse aorta allows f r complete removal of the thrombus. Rethrombosis occurred in the patient on whom we operated, probably because of aortic endarteritis and primary closure of aortotomy. This could have been prevented by use of patch enlargement, as advocated by some authors [2, 31. Regarding the cause, in patient 1, electrocution during pregnancy was the only remarkable event. In patient 2, blood investigations were normal but an inflammatory disease of the aortic intima was found on histologic examination. No aortic obstacle, which could "trap" hypothetical left ventricle thrombi, was seen in these 2 patients. Recently Pozzi and associates [8] reported a case of coarctation with a calcified thrombus at the isthmus. Whether constituted "in situ" or detached from the left heart cavities, thrombus age favors a prenatal disease. In summary, aortic arch thrombosis in the neonate is a rare but highly serious condition of unknown origin, requiring immediate surgical management.

We are mateful to Mrs V. Lecaulain for her secretarial assistance.

References

1. McFaul RC, Keane JF, Nowicki ER, Castaneda AR. Aortic thrombosis in the neonate. J Thorac Cardiovasc Surg 19&4;81: 334-7. 2. Hamilton RM, Penkoske PA, Byme P, Duncan NF.Spontaneous aortic thrombosis in a neonate presenting as coarcation. Ann Thorac Surg 1988;45564-5. 3. Conti VR, Bilfinger TV, Wolf W. Aortic arch thrombosis in the neonate. J Thorac Cardiovasc Surg 1988;9513840. 4. Trowitzsch E, Bemsau U, Luhmer I, Merkle W, Kallfelz HC. Congenital thrombotic odusion of the ascending aorta and the aortic arch. Pediatr Cardiol 1985;6:165-9. 5. Scott WA, Snider AR, Dick M 11, Heidelberger KP, Bove EL, Peschiera AW. Congenital aortic arch thrombosis diagnosed by echocardiography. Am J Cardiol1987;59485-6. 6. Davidson A, Luhmer I, Kallfelz HC. Aortic arch thrombosis in the neonate [Letter]. J Thorac Cardiovasc Surg 1989;97152-3. 7. Conti VR, Wolf W. Aortic arch thrombosis in the neonate [Letter]. J Thorac Cardiovasc Surg 1989;97153. 8. Pozzi M, Taylor J, de Leva1 MR. Coarctation of the aorta associated with calcific aortic thrombosis in a neonate. Eur J Cardiothorac Surg 1991;5:5523.

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Aortic arch thrombosis in the neonate Miguel Sousa Uva, Alain Serraf, François Lacour-Gayet, Jacqueline Bruniaux, Daniel Sidi, Jean Kachaner, Jean-Louis Cloez, Laurent Ferment and Claude Planché Ann Thorac Surg 1993;55:990-992 DOI: 10.1016/0003-4975(93)90132-2

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