Patent Ductus Arteriosus in the Elderly

followed by plasmapheresis on eight occasions was begun. Renal biopsy at this stage showed a crescentic superimposed on a focal and segmental glomerulonephritis. Renal function stabilized with this therap); with creatinine clearance remaining at 32 mVmin with a trace of proteinuria. The chest roentgenogram confirmed resolution of the pneumonia over several weeks, and the Wegener! granulomatosis remained quiescent on a gradually reducing dosage of prednisolone and azathioprine (100 mg daily). The antineubophil antibody level fell during therapy and has remained undetectable for over 12 months, during which time an uneventful sigmoid colectomy has been performed. DISCUSSION

Diagnosis of Wegeners granulomatosis is based upon histologic findings, with other investigations not being helpful. Recently an autoantibody to neutrophil and monocyte cytoplasm has been described which is useful in confirming the diagnosis and monitoring the activity of the disease. I In view of the rapid deterioration seen in this patients condition, a tissue diagnosis was sought by simultaneous lung and paranasal sinus biopsies. The vasculitis was conBrmed, but surprisingly pneumonia due to Legionella pneumophda was also diagnosed, an association not previously described. The decline in renal function during therapy may be attributed to the Legionella pneumonia" or even the erythromycin,' but the glomerulonephritis found on renal biopsy suggests that increased activity of the granulomatous process due to infection was the cause." I( as is often the case, renal rather than lung biopsy had been undertaken to confirm the diagnosis, the patient might well have succumbed to Legionella infection when immunosuppressive therapy was initiated. Our report leads us to suggest that lung biopsy is indicated in the diagnosis of Wegeners granulomatosis, particularly where the radiologic findings are not classical, in order to avoid missing coincidentally associated atypical infection which might progress with the introduction of immunosuppressive therapy ACKNOWLEDGMENTS: We thank the Department of Pathology at Edinburgh University for measurement of the antineutropliil antibody ana the Renal Unit at Edinburgh Royal Infirmary.

REFERENCES 1 Fauci AS, Haynes BF, Katz £ Wolft SM. Wegeners granulomatosis: prospective clinical and therapeutic experience with 85 patients for 21 years. Ann Intern Med 1983; 98:76-85. 2 Van Der Woude FJ, Labatto S, Permin H, van der Giessen M, Rasmussen N, Wtlk A, et ale Autoantibodies against neutrophils and monocytes: tools for diagnosis and markers of disease activity in Wegeners granulomatosis. Lancet 1985;1:425-29 3 Tsai TF, Finn DR, Plikaytis BD, McCauley ~ Martin SM, Fraser

DW Legionnaires disease: clinical features of the epidemic in Philadelphia. Ann Intern Med 1979; 90:509-17 4 Rosenfield J, Gura ~ Boner G, Ben-Bassat M, Livni E. Interstitial nephritis with acute renal failure after erythromycin. Br Med J 1983; 286:938-39 5 Pinching AJ, Rees AJ, Pussel BA, Lockwood CM, Mitchison RS, Peters DK. Relapse in Wegeners granulomatosis: the role of infection. Br Med J 1980; 281:836-38

Patent Ductus Arteriosus In the Elderly* StuartZarich, M.D.; HOWtJrd Leonardi, M.D.;]ohn Pippin, M.D.;

Joseph 7Uthal, M. D.; and Stanley Lewis, M. D.

Patent ductus arteriosus presenting in an elderly patient is unusual. This report describes the oldest patient (72 years) to undergo successful surgical interruption of a patent ductus arteriosus with a unique clinical presentation of typical angina pectoris with normal coronary anatom)' A possible pathophysiologic mechanism for this previously unreported presenting symptom is proposed. The natural history of patent ductus arteriosus and the role of surgery in the elderly patient are discussed. (Chat 1988; 94:110305) pate~t ductu~ arteriosus-is the

third most common congenital cardiovascular anomaly accounting for approximately 10 percent of all cardiac malformations (or roughly 1 in 200 live births). 1 Patent ductus arteriosus is usually diagnosed early in life and treated either by pharmacologic obliteration or surgical interruption; however, there is an increasing awareness of patent ductus arteriosus presenting in adulthood and even in the elderly l-8 In fact, the oldest documented patient with a patent ductus arteriosus lived to 90 years of age. 8 We report a 72-year-old woman successfully treated by surgical division of a patent ductus. To our knowledge, she is the oldest patient successfully treated surgically The clinical presentation of the adult ductus and the role of surgical interruption in the elderly patient are discussed. CASE REpORT A 72-year-old white woman had a several-month history of exertional angina pectoris and increasing dyspnea on exertion. Since childhood a murmur had been noted without associated exercise intolerance. There was no history of acute rheumatic fever or maternal rubella infection. Physical examination revealed a grade 3/6 mid-peaking systolic ejection murmur and an associated grade 2/6 diastolic murmur at the second left sternal border. Findings from pulmonary examination were unremarkable. The electrocardiogram revealed left ventricular hypertrophy The chest x-ray film revealed borderline cardiomegal)t A diagnosis of mixed aortic valvular disease was suspected, and cardiac catheterization was performed. Angiography revealed a normal right dominant coronary arterial circulation. A patent ductus arteriosus was visualized on aortography The calculated pulmonicto-systemic flow ratio was 2.3:1 by oximetry The pulmonary arterial pressures were normal. The left ventricular ejection fraction was 65 percent, with an elevated left ventricular end-diastolic volume index (112 mUm!). Successful surgical interruption of the patent ductus arteriosus was accomplished via a three-clamp technique, thus avoiding cardiopulmonary bypass (Fig 1 and 2~ After surgery the patient reported relief of her exertional chest pain and dyspnea. She has remained alive and free of recurrent symptoms during two years of follow-up.

*From the Cardiology Section, Department of Medicine and Overholt Cardiothoracic Surgical Associates (Department of Surgery), New England Deaconess Hospital and Harvard Medical School, Boston. Reprint requests: Dr. Zarich, Department of CardiologfJ New

EnglandDeaconess Hospital, Boston 02215

CHEST I 94 I 5 I NOVEMBER, 1988

~

1103

Pulmonary Irun

~~~e~~

DC

i~';osu~

:

,,

."

(

.

Vogus n.

,

"

, I

I

I

, , ,

I

I

I

L. S~bcl. or ter y FIGURE 1. Ductal aortic segment is encircled with tapes, and recurrent laryngeal nerve is retracted medially. Note ductalcalcification. Ao, Aorta.

DISCUSSIO:-';

The natural history of a hemodynamically significant patent ductus arteriosus has typically been associated with inexorable cardiovascular derangements, including progressively severe pulmonary hypertension and congestive heart failure . The life expectancy of patients studied has been approximately half that of the general population , with the average age at death being slightly less than 40 years.!? Subsequently, Campbell!' reported greater longevity in untreated patent ductus arteriosus, with 60 percent of the patients surviving to the age of60 years; however, a 4 percent per year attrition rate was noted from the beginning of the fourth decade onward. Nevertheless, progressive pulmonary hypertension is not inevitable in the presence of a persistent patent ductus arteriosus. Actually, significant pulmonary hypertension is relatively uncommon.' It is conceivable that the majority of long-lived patients with patent ductus arteriosus have relatively smalileft-to-right shunts (so-called obstructive ductus vs a wide or hypertensive ductus ") near the level of hemodynamic significance (1.5:1) and, thus, do not overload the pulmonary vascular tree. Other patients may, over time, undergo a spontaneous reduction in the dimensions of the patent ductus arteriosus (as evidenced by a spontaneous closure rate of 0.6 percent per year)." Thus, a small subset of patients survive far longer than previously appreciated. In an adult patient a low index of clinical suspicion coupled with an often atypical murmur can make the diagnosis of patent ductus arteriosus difficult. Only 60 percent of the adults presented with a continuous murmur in a recent series ." The two most common adult presentations are dyspnea and exercise intolerance; however, as many as onethird of adult patients with patent ductus arteriosus are asymptomatic." The etiology of angina in our patient was unclear; however, 1104

a clue may be found in an analogous state of diastolic overload , aortic insufficiency, where the reported incidence of angina pectoris is 3 to 50 percent. '3 Typically, such patients have large left ventricular end-diastolic volumes which could compromise subendocardial perfusion. '4 Our patient also had an elevated left ventricular end-diastolic volume . With exertion the combination of systemic arterial runoff through the patent ductus arteriosus and left ventricular diastolic overload may have resulted in subendocardial hypoperfusion and, thus, ischemia . The relief of angina after surgery supports the contention that the patent ductus arteriosus was causally linked to our patients anginal complaints. The decision to surgically intervene in an elderly patient will be influenced by many factors. A patent ductus is frequently complicated by aneurysmal dilatation of the ductus, ductal as well as aortic calcification, and pulmonary hypertension, which hinder surgical success. Thus, nonsurgical closure using a catheter-inserted synthetic plug may be a therapeutic alternative to thoracotomy. is In an effort to ensure safe surgical interruption of a complicated ductus, numerous innovative surgical approaches have been proposed;'·'8-'" however, the aforementioned approaches may be unnecessarily complex for the elderly patient with patent ductus arteriosus. Our surgical technique emphasizes ductal disconnection without the use ofcardiopulmonary bypass. John et all. employed an identical three-clamp technique in 128 patients and similarly avoided the need for cardiopulmonary bypass in all but three patients. Recently, long-term results of surgically and medically treated adult patients with patent ductus arteriosus were reported. ~ Over a mean duration of 19 years, the surgical survival rate was significantly better than the medical survival in the nonsurgical group (93 percent vs 83 percent). Sixty percent of the surgically treated patients reported

FIGURE 2. A, Closure of aortotomy follows ductal disconnection. Gentle rotation of aorta towards surgeon facilitates closure. B, Ductalclosure completedafter releaseofaorticcross-elamps.

PDA in the Elderly (Zarich sf 81)

improved symptoms, while fewer than 20 percent of the medically treated patients improved. Although severe pulmonary hypertension and cardiomegaly portended a poorer prognosis, there were no deaths in patients with peak pulmonary pressures less than 70 mm Hg. These encouraging results led these investigators, as well as others, 7.11 to recommend closure of a patent ductus arteriosus at the time of diagnosis, unless a right-to-left shunt was present Our report reemphasizes that patent ductus arteriosus is compatible with survival to advanced age, even though the natural history of this anomaly usually portends significant morbidity and premature death. Surgical repair to improve symptoms, reduce morbiditg and prolong life expectancy should not be withheld (even in certain subsets of patients with moderate pulmonary hypertension) unless there is firm evidence that the ductus is closing. 7.8.11. The surgical techniques currently employed have made interruption of the patent ductus arteriosus in the elderly patient safer; and, thus, age alone in the absence of a right-to-Ieft: shunt or severe pulmonary hypertension should not be viewed as a contraindication to surgery ACKNOWLEDGMENTS: We thank Ms. Dolores Fagan and Ms. Ann Erickson for secretarial assistance in the preparation of this manuscript REFERENCES

1 Kelly D. Patent ductus arteriosus in adults. Cardiovasc Clin 1979; 10:326 2 Homsten 1: Hellerstein H, Arkeny J. Patent ductus arteriosus in a 72-year-old woman. JAMA 1967; 199:148-50 3 WoodrufF ~ Gabliani G, Grant A. Patent ductus arteriosus in the elderly South Med J 1983; 76:1436-37 4 Aiken J, Bifalco E, Sullivan JJ. Patent ductus arteriosus in the aged. JAMA1961; 177:330-31 5 Bell-Thomson J, Jewell E, Ellis FH, Schwaber JR. Surgical technique in the management of patent ductus arteriosus in the elderly patient. Ann Thorac Surg 1980; 30:80-3 6 Syrkis I, Machtey I, Sagiv M. Patent ductus arteriosus in old age. J Am Geriatr Soc 1975; 23:333-36 7 Marquis RM, Miller H, McCormack R, Matthews M, Kitchin A. Persistence of ductus arteriosus with left to right shunt in the older patient Br Heart J 1982; 48:469-84 8 Fisher RG, Moodie D, Sterbar, Gill C. Patent ductus arteriosus in adults-long-term follow up: nonsurgical versus surgical treatment J Am Coli Cardioll986; 8:280-84 9 Erdman S, Levinsky L, Levy M. A simple method for closure of patent ductus arteriosus in elderly patients. Ann Thorac Surg 1979; 27:84-8 10 Key A, Shapiro M. Patency of the ductus arteriosus in adults.

16 17

18 19

ductus arteriosus: experiences in 61 consecutive cases treated without thoracotom): Circulation 1975;51:337-41 Robinson G, Condit D. Attai A. Surgical treatment of difticu1t patent ductus arteriosus: profound hypothermia and circulatory arrest. NY State J 1975; 75:2190-93 18ira A, Akita H. Patch closure of the ductus arteriosus: an improved method. Ann Thone Surg 1976; 21:454-57 Gold J~ Cohn LB. Operative management of the calcified patent ductus arteriosus. Ann Thone Surg 1986; 41:567-71 John S, Muralidharan S, Jaivaj ~ Mani G. Babuthaman, DrishnaswamyS, et ale The adult ductus: review ofsurgical experience with 131 patients. J Thorac Cardiovasc Surg 1981; 82:314-19

Magnetic Resonance Evaluation of Patency of Stented Polytetrafluoroethylene Graft Connecting Right Atrium to Pulmonary Artery* SugGto NGWfJ. M.D.; Ma1coto ytmaatla, M.D.; HitOflU/d Iris, M.D.; and Shigeru 7Bnmaoto, M.D., EC.C.I!

Magnetic resonance imaging (MRI) was used to evaluate late postoperative patency of a Rented polytetra8uoroethy1ene (PTFE) graft, which had been used to CODDect the right atrium to the pulmonary artery in a patient with tricuspid atresia. The MRI depicted patency, luminal size, course, and geometric morphology of the conduit. (Claat

1988; 94:1105-(1)

,ls the operative criteria for the Fontan procedure have been expanded.t> prosthetic conduits are often used today to connect the right atrium to the pulmonary arte~3 though the exclusive utilization of the native tissues is recommended.' One of the most important problems with the prosthetic materials is the long-term patency of the conduit used in the Fontan procedure. The patency of such grafts has been evaluated by invasive angiocardiograph)t Magnetic resonance imaging (MRI) may distinguish rapidly 80wing blood within grafts and/or vessels from nonvascular structures without injecting contrast media, and the evaluation may be repeatedly performed with outpatients. We used MRI to assess the patency luminal size, course, and geometrical morphology of the externally spirally stented polytetralluoroethylene (PTFE) graft (Impra-Flex graft) used in the Fontan procedure.

1'1

Am Heart J 1943; 25:158-65

11 Campbell M. Natural history of persistent ductus arteriosus. Br Heart J 1968; 30:4-13 12 Nugent EW The patholo~ abnormal physiolo~ clinical recognition and medical and surgical treatment of congenital heart disease. In: The heart New York: McGraw-Hill Book Co, 1986:614-18 13 Goldschlager N, Pfeifer J, Cohn K, Popper R, Selzer A. The natural history of aortic regurgitation. Am J Med 1973; 54:57784

14 Basta L, Raines D, Najjar S, Kioschos JM. Clinical haemodynamic and coronary angiographic correlates of angina pectoris in patients with severe aortic valve disease. Br Heart J 1975; 37:150-57 15 Sato K, Figino M, Kozaka 1: Thmsferral plug closure of patent

Moo

CASE REPORT

A 14-year-old boy with bicuspid atresia underwent a modi&ed Fontan procedure. The patient had received the superior vena cava right pulmonary artery anastomosis (modi&ed Gleeni shunt) at the age of one year and three months. At operation, the diameter of the pulmonary trunk was 40 percent of the normal diameter; the pulmonary trunk was too small to directly anastomose to the right atrial appendage. Accordingl~ an Impra-Flex graft (19 mm in diameter) was used to connect the right abium to the pulmonary *From the Second Department of

Surge~

Obyama University

Medical School, Olcayama, Japan. &print f8quut8: Dr: NtIWtJ, SBcond DBp!Jrtment of S~ery, Olcarama UnitJBrBUy MedJcal School, 2-5-1 ShiIcattJ-chO, OktiymraG 700,

Japan

CHEST I 94 I 5 I NOVEMBER. 1988

1105