ABSTRACT Spontaneous aortic thrombosis in the neonate is a rare entity of uncertain etiology with a high mortality. To our knowledge, this is the first report of a newborn surviving surgical treatment of spontaneous thrombosis of the thoracic aorta.
Aortic thrombosis in neonates is rare and usually related to umbilical artery catheterization. Spontaneous aortic thrombosis in a neonate has been described . The location is more commonly abdominal than thoracic. Management has been varied and usually unsuccessful. Spontaneous thrombosis of the thoracic aorta has been lethal in the cases reported [4, 51. We report the case of a newborn with spontaneous thrombosis of the thoracic aorta who was treated surgically and survived.
This 2,810-gm, 38-week-gestation male infant was born to a 33-year-old gravida I1 para I mother. Prenatal history was normal until the day of delivery when she was seen with absent fetal movements. Emergency cesarean section was performed. The infant had marked respiratory depression, with Apgar scores of 4 and 8, and required resuscitation.
The skin appeared mottled but pink. Heart rate was 130 beats min, respiratory rate was 64/min, and blood pressures were: 106194 mm Hg in the right arm, 84/66 mm Hg in the left arm, 36/25 mm Hg in the right leg, and 48/31 mm Hg in the left leg. Precordial impulse was increased, and the liver edge was 3 cm below the right costal margin. The left femoral pulse was palpable but decreased. The right femoral pulse was not palpable. A Grade 216 systolic ejection murmur was audible at the heart base. Umbilical arterial catheterization had not been performed. Arterial blood gases were pH 7.27, carbon dioxide tension was 48.5 mm Hg, oxygen tension was 37 mm Hg, and hematocrit was 66.5%. Chest radiograph showed moderate cardiomegaly and mild pulmonary plethora.
Two-dimensional echocardiography demonstrated a structurally normal heart but the almost complete narrowing of the aorta just above the level of a persistent ductus arteriosus. Left ventricular contractility was markedly reduced, with a shortening fraction of 0.13 (normal, 0.2tL0.50). An infusion of prostaglandin El, 0.05 mg/kg/min was started. Cardiac catheterization was performed. Left ventricular pressure was 86/12 mm Hg, and the descending aortic pressure was 32/26 mm Hg. Figure 1 shows the posteroanterior view of the aortic arch from the left ventricular angiogram. There was the obstruction in the right subclavian artery at the level of its exit from the thorax and a severe stenosis of the left the subclavian artery with a thrombus within this vessel. The lateral view (Fig 2) demonstrated an obstruction to flow distal to the left subclavian artery.
In the operating room, the aortic arch was visualized through a left-sided thoracotomy. The aorta was opened and an organized, partially adherent thrombus was removed. The thrombus originated just distal to the left carotid artery and sent a tail into the left subclavian artery, which was hypoplastic. The ductus arteriosus was patent, with no thrombus in its lumen. There was no visible external coarctation or posterior shelf internally. The aorta was closed with a Gore-Tex patch to prevent narrowing from surgical scamng at the incision line. Pathological study demonstrated a clot measuring 6 x 5 x 4 mm that consisted of fibrin and foci of calcification.
The platelet count fell gradually to 34,000/~1 on the fourth postoperative day but then returned to normal. Prothrombin time was 13.1 to 13.5 seconds (normal, 1S30 seconds). Partial thromboplastin time was 45 seconds on the second postoperative day (normal, = 46-65 seconds). Protein C and antithrombin I11 levels were below normal: protein C was 0.45 units (normal, 0.63-1.27 units) and antithrombin I11 was 0.47 units (normal, 0.75-1.25 units).
Postoperatively, the left femoral pulse was normal. Signs of congestive heart failure resolved quickly. The right femoral pulse did not return, and an ischemic area developed on the tip of the right small toe. The rest of the limb was pink and well perfused. The right arm was intermittently cyanosed, and the pulse was absent. Heart size returned to normal by the fifth postoperative day. The infant has been followed up for six months and is developing normally and is receiving no medications.
Author: Dr. Patricia Penkoske
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