Hemothorax

Clare M. Laroche M.D.; Francis Wells, M.D.; and John Shneerson, D.M.

A 37-year-old woman with hereditary telangiectasia suffered a life-threatening hemothorax due to an enlarging pulmonary arteriovenous malformation in pregnancy. This was treated by emergency right lower lobectomy and excision of arteriovenous malformations in the right middle and upper lobes, with no postoperative complications, and the subsequent delivery of a normal infant. Women with hereditary telangiectasia contemplating pregnancy should be screened for the presence of PAVM to anticipate complications.

Several hundred cases of PAVM have been reported in the literature,[1] but the natural history of this rare condition is still poorly understood. About one half of the cases are associated with hereditary telangiectasia (Osler-Weber-Rendu disease), while the remainder are usually isolated. The mortality of untreated PAVM is significant. One study of 27 patients found an 11 percent mortality over six years, with 26 percent experiencing significant morbidity.[2] Pregnancy has been reported to increase the size of PAVM,[3,4] but only brief details of two cases of massive hemothorax during pregnancy have previously been recorded.[5] A recent report[6] described a hemothorax occurring in a pregnant woman with previously unrecognized PAVM due to hereditary telangiectasia. We report a patient with known hereditary telangiectasia, who at 29 weeks' gestation, developed a massive hemothorax from a PAVM, requiring thoracotomy.

CASE REPORT

At the age of 14, a shadow was detected in the patient's left upper lobe on a chest roentgenogram (Fig 1), performed because of a positive Heaf test. Anti-tuberculosis chemotherapy was given, following which the shadowing was thought to have resolved, and she subsequently underwent annual review. At the ages of 21 and 25, she suffered spontaneous abortions both at ten weeks' gestation, following which she was investigated for secondary infertility, but no abnormality was found. At age 31 numerous small red lesions were noted on the mucous membranes of her mouth, palms of her hands, and under her finger nails. Other members of her family on her father's side were found to have had the same features. At the age of 36, she suffered severe menorrhagia, and was treated with oral iron for anemia (Hb, 7.3 g/dl). Six months later, she was found to be polycythemic with a Hb value of 18.2 g/dl (packed cell volume, 0.55) and had central cyanosis with marked clubbing of both finger and toe nails. She had no clinical or electrocardiographic evidence of right ventricular hypertrophy. However, chest roentgenogram showed numerous opacities throughout both lung fields, which were shown by pulmonary angiography to be PAVM, the largest being in the left upper lobe. Polycythemia persisted, and she subsequently underwent balloon embolization of the largest lesion, with an improvement in her resting arterial oxygen saturation from 85 to 93 percent. One year later, her exercise tolerance had increased and her polycythemia and clubbing had resolved. She again became pregnant, and at 24 week's gestation, new telangiectasia on her tongue and hands were noted. At 29 weeks, she suddenly developed dyspnea, cough, and right-sided pleuritic chest pain, with signs of a large pleural effusion. Pleural aspiration revealed a hemothorax.

Repeat pulmonary angiogram showed numerous AV shunts, too extensive for embolization (Fig 2). Further intrapleural hemorrhage led to severe dyspnea and circulatory collapse and she subsequently underwent emergency right lower lobectomy and excision of AV malformations in the right middle and upper lobes. There were no postoperative complications apart from a profuse epistaxis which required cautery. Two months later, at the age of 39 years, she delivered a healthy male infant by cesarian section. She remains well and has had no further intrathoracic hemorrhages in the three years of follow-up.

DISCUSSION

Most cases of PAVM are congenital, but the condition is often not recognized until the second decade.[7] The PAVM progressively enlarge with age, probably in response to increased blood flow, with eventual necrosis of the vessel wall. These effects may increase the magnitude of the shunt and worsen systemic hypoxia, or may cause hemorrhage, leading to clinical detection.[6] Hemothorax is a rare complication of PAVM and is potentially fatal.[8]

The effects of pregnancy on PAVM have only been recorded previously in four patients. Two patients were known to have hereditary telangiectasia: in one patient, three PAVM were noted on routine chest roentgenogram ten months after the delivery of her first child, although they had not been visible roentgenographically two years previously;[3] in a second woman, with known PAVM, pregnancy had to be terminated at 35 weeks' gestation due to life-threatening hypoxia.[4] Two patients with recognized PAVM developed massive hemothorax during the fifth and eighth months of pregnancy,[5] but no further details of these patients are available. A recent report[6] described a woman with unrecognized hereditary telangiectasia who suffered a spontaneous hemothorax in the 24th week of pregnancy, and who underwent successful coil spring embolotherapy for four PAVMs, with the delivery of a live infant at 30 weeks' gestation.

Our case documents a patient with known hereditary telangiectasia, who developed an increase in size of known PAVM during pregnancy, with the development of massive hemothorax. It was not possible to perform repeat balloon embolization in this patient, both because of her serious condition, and because of the size and number of PAVM. Surgical resection in nonpregnant patients is not only safe, but also effective,[9] although repeated resections may lead to dyspnea and even right heart failure due to loss of lung tissue.

Increased growth of PAVM during pregnancy may be due to the increased cardiac output, to hormonal effects on the blood vessels, or both. Blood volume and cardiac output increase from 8 to 30 weeks' gestation, and although overall pulmonary artery pressure is little affected, this may give rise to increased flow through the low-resistance pathway of the AVM. In addition, the increased level of progesterone which causes relaxation of arteriolar smooth muscle, may also cause dilatation of vessels within the PAVM, reducing their resistance and further increasing blood flow through the PAVM.[10]

Since about 15 percent of patients with hereditary telangiectasia have coexisting PAVM, all women with the condition who contemplate pregnancy should at the very least have a chest roentgenogram to exclude PAVM. In pregnant women with known PAVM, chest roentgenogram and arterial blood gases or oximetry should be performed at four and six months' gestation in order to anticipate complications.

*From the Department of Chest Medicine, Newmarket General Hospital, Newmarket, Suffolk, and the Surgical Unit, Papworth Hospital, Papworth Evarard, Huntingdonshire, UK.

REFERENCES

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10 Elliott JA, Rankin RN, Inwood MJ, Milne JK. An arteriovenous malformation in pregnancy: a case report and review of the literature. Am J Obstet Gynecol 1985; 152:85-88

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