False Aneurysm of A Palliative Shunt Producing Massive Hemoptysis

FICURE 3. The extracted Beall mitral valve prosthesis demonstrates no evidence of strut malfunction as the cause of the missing disc.

on the ventricular side of the disc has heen reported using eehocardiography.P M-mode echocardiographic patterns of the Beall valve mitral prosthesis have heen previously described.v but two-dimensional echocardiographic studies of this valve have not heen reported. Our report demonstrates the usefulness of M-mode and two-dimensional echocardiography in the identification of an extruded Beall mitral valve disc into the left ventricle. While cardiac catheterization was performed in our patient, we believe that such a diagnostic procedure with its increased risk in a critically ill patient was not necessary in light of the diagnostic features demonstrated on the echocardiographic studies. Our patient documents another case of a survivor from prosthetic disc embolization. Unfortunately, the location of the disc in this patient was never discovered and it is possible that it fractured into small fragments that did not permit localization. This report also points out the potential importance of two-dimensional echocardiography at the bedside as a means of evaluating sudden complications from prosthetic valve dysfunction . ACKNOWLEDGMENT: The authors wish to express their appreciation to Ms. Connie Parrotto and Ms. Linda Meixell for technical assistance.

fu:FEnENCES 1 Hughes DA, Leathennan LL, Nonnan ]C, Cooley DA. Late embolization of prosthetic mitral valve occluder with survival following reoperation. Ann Thorac Surg 1975; 19:212-15 2 Ansbro ] , Clark R, Gerbode F. Successful surgical correction of an embolized prosthetic valve poppet. ] Thorac Cardiovasc Surg 1976; 72: 130-32 3 Roberts AK, Lambert C], Mitchel BF. Embolization of disc occluder of a Wada-Cutter mitral prosthesis with survival. Ann Thorac Surg 1976; 21:361-64

110 CAFFARENA, LLAMAS, mRD-COTO

4 BeaI1 AC ]r, Bloodwell RD, Liotta D, Cooley DA, DeBakey ME. Clinical experience with a Dacron velour covered Teflon disc mitral prosthesis. Ann Thorac Surg 1968; 5:402-10 5 Brawley RK, Donahoo IS, Gott VL. Current status of the Beall, Bjork-Shiley, Braunwald-Cutter, Lillehei-Kaster and Smeloff-Cutter cardiac valve prostheses. Am ] Cardio11975; 35:855-65 6 Beall AC. Late results with cardiac valve replacement. Reduction of thromboembolic complications of mitral valve replacement. ] Cardiovasc Surg 1972; 13:261-67 7 Robinson M], Hildner F] , Greenberg JI. Disc variance of Beall mitral valve. Ann Thorac Surg 1971; 11:11-16 8 Burch GE, Giles TD. Clinical Evaluation of aortic and mitral valve prostheses. Am Heart ] 1976; 92:245-51 9 Keen G. Late death due to escape of ball from mitral valve prosthesis. ] Thorac Cardiovasc Surg 1974; 67: 202-07 10 Bonnabeau RC], Lillehei CWo Mechanical ball failure in Starr-Edwards prosthetic valves. ] Thorac Cardiovasc Surg 1968; 56:258-64 11 Nathan M]. Strut fracture. A late complication of Beall mitral valve replacements. Ann Thorac Surg 1975; 16: 610-13 12 Reid ]A, Stevens TW, Sigwart U, Fulweber RC, Alexander ]K. Hemodynamic evaluation of the Beall mitral valve prosthesis. Circulation 1972; 45:1-7 13 Oliva PB, Johnson ML, Pomerantz M, Levene A. Dysfunction of the Beall mitral prosthesis and its detection by cineHuoroscopy and echocardiography. Am ] Cardiol 1973; 31:393-96 14 Stanford W. In discussion of systemic embolism and the Beall mitral valve prosthesis. Ann Thorac Surg 1970; 10:20-26 15 Johnson ML, Holmes ]H, Paton BC. Echocardiographic determination of mitral disc valve excursion. Circulation 1973; 47:1274-80

False Aneurysm of A Palliative Shunt Producing Massive Hemoptysis* Jose Marla CafJarena, M.D.; Patricio Llamas, M.D.; and Eduardo Otero-Cato, M.D. An 18-year-old woman suffered repeated episodes of IlUlSSive hemoptysis. Radiographic findings suggested the possible presence of an aneurysm of the aortic arch, bnt aortography was negative. A palliative shunt, with a Tefton conduit interposed between the subclavian and left pulmonary arteries, had been constructed in 1964, and total correction including ligation of the shunt was performed in 1969. At operation, in 1980, a false anenrysm of the systemic anastomosis of the shunt was found. This complication might appear in other patients in whom prosthetic conduits were nsed for palliative shunting.

A

large number of complications of palliative surgery have heen reported.' Hemoptysis may appear after operation for tetralogy of Fallot in patients with enlarged collaterals, pulmonary hypertension or other °From the Servicio Cirugia Cardiovascular, Ciudad Sanitaria "La Fe," Valencia, Spain.

CHEST, 81: 1, JANUARY, 1982

secondary vascular changes." The following is a report of another cause, not previously described to our knowledge, of hemoptysis after palliation with an unusual type of systemic-pulmonary shunt. CASE REPoRT

An 18-year-oJd woman was admitted for surgical treatment of recurrent severe hemoptysis. She had been found to have congenital heart disease at one month of age and, because of development of hypoxic spells, a palliative shunt was constructed when she was two years old by interposition of an 8 mm Teflon conduit between the left subclavian artery and the left pulmonary artery. Marked improvement ensued, with disappearance of cyanosis. In 1969 she was sent to another hospital, where cardiac catheterization was performed and revealed right ventricular systemic pressure and right-to-left shunt at the ventricular level. Angiocardiography showed a large ventricular septal defect, small pulmonary artery, severe infundibular stenosis, aberrant origin of the right subclavian artery, and a well functioning subclavian-pulmonary shunt. Total correction of the anomaly was performed in October, 1969, including suture closure of the ventricular septal defect with a Dacron patch. infundibulectomy, pulmonary valvotomy and ligation of the subclavian-pulmonary shunt. She remained asymptomatic until May, 1980. when she suddenly suffered massive hemoptysis with hypovolemic shock. She recovered and suffered 15 new episodes of hemoptysis, six of volume greater than 600 ml. At that moment, physical examination showed a grade 1/6 systolic murmur at the third left intercostal space, and normal pulses in all extremities . Coagulation and other analytical studies were normal. The chest x-ray film initially showed an irregular shadow of homogeneous density in the left upper lobe and mediastinum; later on, a rounded mass was clearly seen in the area of the aortic arch (Fig 1 and 2) . Bronchoscopic examination showed the origin of bleeding in the left upper lobe, but failed to show the cause. Cardiac catheterization was performed and showed adequate repair of the anomaly with a small (17 mm Hg) residual gradient in the right ventricular outflow tract. Angiocardiography did not show any intracardiac defect or valve malfunction; the aortic arch appeared normal, without evidence of aneurysm or dissection. The patient was referred to our hospital and operation (left thoracotomy) was performed in July, 1980, under cardiopulmonary bypass and general hypothermia at 14°C. The left femoral artery was used for arterial return, and the left femoral vein and the left atrium for venous drainage. After section of the adherences, the apex of the lung was exposed. The internal. surface of the left upper lobe was adherent to a thrombosed pseudoaneurysm, about 6 em in diameter, found at the level of the systemic anastomosis of the shunt. Lateral clamping of the left subclavian artery allowed safe resection of the pseudoaneurysm and part of the conduit. The orifice in the subclavian artery and the distal end of the Dacron conduit were oversewn with running sutures. Closure of bronchial openings and obliteration of the residual lung cavity were finally done. The patient recovered well and was discharged after an uneventful 14-day postoperative course. Four months later, she is asymptomatic, leading a normal life.

CoMMENTS False aneurysms have been reported in association

CHEST, 81: 1, JANUARY, 1982

FxCURE 1. Posteroanterior chest film showing widening of the upper mediastinum produced by an abnormal shadow present in the left upper border.

with thrombosed prosthetic grafts," essentially the same condition present in our patient. Rapid development of the aneurysm 16 years after surgery seems unusual, and may suggest infection, but no symptoms or surgical or bacteriologic evidence of infection was present. Silk sutures were used at operation and dissolution of suture lines may have been responsible for formation of the aneurysm. Although a patent graft is not a prerequisite for the formation of anastomotic false aneurysms.s the

FxGURE 2. Tomography showed the ezistence of a rounded, smooth. well-defined soft tissue mass in relation to the aortic arch.

FALSE ANEURYSM PRODUCING MASSIVE HEMOmSIS 111

suture line must be in contact with the blood stream, as happens in shunts in which the vessels shunted remain patent after closure of the prosthetic shunt. Similar aneurysms might, therefore, appear in other types of palliative procedures involving the use of conduits and closure of the anastomotic stoma may be preferable to simple ligation of the shunt. Changes in suture material, however, have been effective in markedly decreasing suture failure as a cause of anastomotic aneurysms,' making this complication improbable.

REFERENCES 1 Weldon CS. Complications of systemic pulmonary artery shunts. In: Complications of intrathoracic surgery, Cordell AR, Ellison RG, eds. Boston: Little, Brown, 1979 2 Haroutunian LM, Neill CA. Pulmonary complications of congenital heart disease: Hemoptysis. Am Heart J 1972; 84:540-59 3 Sumner DS, Strandness DE. False aneurysms occurring in association with thrombosed prosthetic grafts. Arch Surg 1967; 94:360-62 4 Moore WS. Anastomotic aneurysms. In: Vascular surgery, Rutherford RB, ed. Philadelphia: WB Saunders, 1971

Alphal-Antitrypsin Deficiency and Common Variable Hypogammaglobulinemia in a Patient with Asthma * NgUf/en D. Phung, M.D.;t Ralph T. Kubo, Ph.D.;: and Sheldon L. Spector, M.D., F.C.C.P.§

Severe alphal....titrypsia deficiency with pheootype PIZZ was fOUDd In 8IIOdatioD with COmmoD VIIIi8bIe hypopmmaglobullaemia and hyperreactive llirways . . ease in a 34-year-old patieDt. Immunologic studies de.oostrated normal ceDuiar ImmDDity but impaired respGDSeS to iDftueoza and ,DeamococCIII 8IltIIe... IovestiptiODS of the mechanism of h _ b a B Demia revealed DO iDtriDsic B ceO defect, syotlaesls abnormality, or Impairment of secretioD of Imm_oglobuIiDs. A serum suppressive factor was preseDt aDd probably accounted for the hypopmmagloballaemia.

is a protease inhibitor that beA lphal-antitrypsin lieved to play an important role in the pathogenesis is

of emphysema," Severe deficiency of this protein, often associated with phenotype 1:L or SZ, leads to clinical emphysema in early adulthood.f Common variable hypogammaglobulinemia is a heterogeneous group of disorders," frequently associated with recurrent sinobronchopulmonary infections, bronchiectasis, gastroin-

=

-From the National Jewish Hospital and Research Center and the University of Colorado Medical School, Denver. enow in Allergy-elinical Immunology. · t Professor in Microbiology and Immunology. §Senior Staff Physician. Reprint requests: Dr. Spector, National Jewish H08fJital and Research Center, 3800 East C ai/ax, Denver 80206

112 PHUNG, KUBO, SPECTOR

testinaI abnormalities, and a high degree of susceptibili-

ty to neoplasia.' There is no known association between immunoglob-

ulin deficiency and Al-antitrypsin deficiency. We describe a patient with Cll-antitrypsin deficiency, common

variable hypogammaglobulinemia, hyperreactive airways disease, and the Zl phenotype. CASE REPORT

The patient was a 34-year-old man in good health until 1976, when progressive dyspnea on exertion developed. An open lung biopsy in March 1977 showed panacinar emphysema. Severe At-antitrypsin deficiency with phenotype ZZ was then diagnosed. After referral to National Jewish Hospital, his history of frequent respiratory tract infections, wheezing, and rhonchi prompted further investigation. Both panhypogammaglobulinemia and hyperactive airways disease were documented. The latter diagnosis was confirmed by a positive histamine and methacholine inhalation challenges and favorable response to bronchodilators. He had smoked minimally (four packs a year) for three years, stopping several years before admission. He was retired and had worked with paint and plywood. His family history was remarkable in that his mother had an Al-antitrypsin phenotype SZ and chronic bronchitis. His father was asymptomatic, with MZ phenotype. A paternal uncle died of cancer of the esophagus at the age of 40 years, with emphysema diagnosed at autopsy. A paternal cousin was found to have 40 mg/dl At-antitrypsin. The patient had no siblings and no children. Pertinent findings on physical examination included dry rales at the lung bases and early bilateral posterior subcapsular cataracts. A chest x-ray film showed pleural tenting on the right side and platelike atelectatic changes in the left mid-lung Beld, A sinus x-ray film showed a softtissue density of the inferior floor of the left maxillary. Pulmonary function testing showed the following results: hyperinHation of the lung with a vital capacity of 5.74 L (114 percent of normal) , residual volume of 5.28 L (328 percent predicted) and total lung capacity 11.02 L (158 percent predicted). Flow rates showed airway obstruction with FEV 1 of 2.26 L (5 i percent predicted), FEVt/FVC ratio of 43 percent, and FEF 25-751 0.82 L (14 percent). Specific conductance was decreased at 0.06 L/em H.,D/L/sec (26 percent predicted). Diffusing capacity ( Dco) was 13.8 ml/min/mm Hg (42 percent predicted). The methacholine bronchial inhalation challenge was positive, with a 36 percent fall of FEV 1 after Bve breaths of 0.31 mg/ml. The histamine bronchial inhalation challenge also was positive, with a 27 percent fall in FEV 1 after five breaths of 2.5 mg/ml, . MATERIALS AND METHODS

Determination of IgG, IgA, IgM, AI-antitrypsin levels, P t phenotyping, enumeration of T and B cells, and in vitro lymphocyte stimulation studies with phytohemagglutinin and pokeweed mitogen (PWM) were done with standard techniques. Cytoplasmic immunoglobulins and immunoglobulin biosynthesis studies were done as previously deseribed.s Specific Antibody Response Studies

Isohemagglutinins were measured by standard hemagglutination inhibition techniques. The patient was given a

CHEST, 81: 1, JANUARY, 1982