Anomalous Pulmonary Venous Connection

----iAr----slleCled reports

Anomalous Pulmonary Venous Connection· Medical Therapy

Dana G. Kluner, M.D., F.C.C.P. ; and Richard P. Sorkin, M.D.

Anomalous pulmonary venous connection is a congenital defect which may 6nt be diagnosed in the adult who has developed lung disease or pulmonary vascular obstructive disease. Surgical repair may be inadvisable in such a case. 1b date, little attention has been paid in the literature to medical management. A case is presented in which medical palliation with nitrate therapy was successful.

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he term "anomalous pulmonary venous connection" (APVC) refers to a variety of congenital defects in which some or all pulmonary venous blood returns to the right abium. Pulmonary veins may connect to the right atrium, the coronary sinus, the venae cavae or their tributaries. Associated atrial septal defect (ASD) is common. APVC is not rare, having been found in 0.4 to O. 7 percent ofadult cadavers autopsied. 1 Clinically, it is less frequently recognized because physiologic significance varies widely and depends on several factors : presence of other major cardiac defects, presence and size of an associated ASD, number of pulmonary veins draining anomalously, presence and degree of obstruction to pulmonary venous return, and the response of the pulmonary vasculature. Additionally, APVC may be unmasked and made significant in the previously asymptomatic adult by lung resection or disease, especially if normally draining parts of the lung are affected. I Definitive therapy for APVC is surgical repair. Medical management is reserved for pre-operative and inoperable patients. Very little attention has been paid in the literature to the details of medical management, with only passing references being made to therapy with digitalis and diuretic agents." We present the case of an adult whose partial APVC both complicated and was complicated by severe lung disease and previous resection of a normally draining lobe . Because she was a poor surgical candidate, she was treated medically. This is the first report of the use of vasodilator drugs to successfully palliate symptoms due to APVC complicated by lung disease.

history included pulmonary tuberculosis, treated with chemotherapy from 1960 to 1963, and a right upper lobectomy and thoracoplasty in 1961. She had never smoked. On physical examination she appeared cyanotic, edematous and in severe respiratory distress. Blood pressure was130nO mm Hg, pulse l20/min and regular, and respiration rate 6O/min. Neck veins were distended. There were diffuse rales. The precordium was hyperdynamic with a parasternal heave and a displaced point of maximum impulse. The second heart sound was accentuated, and a right ventricular third heart sound was present. A grade 3/6 systolic murmur was audible throughout the precordium. The liver was enlarged and pulsatile. Right atrialabnormality and right ventricular hypertrophy were present on ECG examination. Chest x-ray film is shown in Figure 1. Arterial POI was 41, and Pcoj, 35. Initial therapy consisted of oxygen, furosemide and the application of 2 inches of 2 percent nitroglycerin ointment every 4 hrs . By the third hospital day, she had lost 6 kg of 8uid and appeared markedly improved. Cardiac catheterization was performed on day 4 while the patient was on the same medical regimen. Hemodynamic data and oxygen saturation levels are presented in 'Iable 1. A catheter was easily passed from the right atrium into a massively dilated superior vena cava, and from the right atrium across a high inter-atrial septal defect consistent with a sinus venosus ASD . Contrast injections into the left atrium demonstrated trivialleft-to-right shunting across the ASD . Injections into the right atrium demonstrated venous blood from the entire left lung draining into a large vertical vein which joined the left brachiocephallc vein (Fig 2). The right lung drained normally. Right heart output was calculated to be 8.2 Umin by thermodilution technique. Based on oximetry data, there was a 2:1 left-to-right shunt. Left atrium and ventricle were small, and left ventricular function was normal. On day 8, nitrate therapy was discontinued. The next day, despite

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CASE REPORT A 47-ye&I"Old gravida 9 para 9~man presented in 1981with a twomonth history of progressive dyspnea and edema. Past medical ·From the Departments of Internal Medicine, Divisions of Pulmonary and Critical Care Medicine, Wayne County General Hospital, Westland, Michigan; University ofMighigan Medical Center. Ann Arbor: and Division of Internal Medicine, Section of Cardiology, Lutheran General Hospital, Park Ridge, Illinois . &mint f'fJquutI: Dr: Kimler; 3688 KamplighterDrioe, Ann Arbor,

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FIGURE 1. Posteroanterior chest x-ray film. Surgical changes from previous thoracoplasty and lobectomy, calcifications and scarring from old cervical and pulmonary tuberculosis, pulmonary vascular congestion, cardiomegaly, and "figure eight" configuration of superior mediastinal and cardiac shadows suggestive of APVC are seen. Anomalous Pulmonary

venous Connection (Kissner, Sorldn)

FIGURE 2. Contrast injection into the right ventricle . PA,pulmonary artery; V. vertical vein draining left lung; A, aorta; B, brachiocephalic vein; SVC, massively dilated superior vena cava. no weight gain, she was again in severe respiratory distress due to pulmonary edema. Reinstitution of topical nitrate therapy led to prompt and marked Improvement. A trial treatment with hydralazine proved unsuccessful due to resultant hypotension. She was discharged on therapy with isosorbide dinltrate 60 mg every 4 hrs orally, furosemide 80 mglday, and oxygen 24 hrsIday. On this program she was able to resume her previous activities, including housework. Pulmonary function tests performed when there was no evidence of heart failure demonstrated a severe restrictive defect with FVC of 0.86 L (27 percent of predicted), FEV. of 0.76 L (32 percent of predicted) and TLC 2.1 L (45 percent of predicted). She did very well until the spring of 1984, when she developed progressive respiratory insufficiency with hypercapnia, leading to her death in April, 1985. Permission for autopsy was not granted. DISCUSSION

Anomalous pulmonary venous connection results in a leftto-right shunt with increased pulmonary blood flowand right ventricular dilitation. As in ASD, pulmonary vascular resistance may be normal initially, unless there is obstruction to pulmonary venous return. However, pulmonary vascular obstructive disease may develop as a later complication, even Table l-Cardiac Catheteriz.ation % Oxygen

Site

saturation

SVC IYC mldRA mldRV PA PCW LA LV Aorta

84 75 86 81 83 90 91 91

Pressure (mm Hg)

7 6010 to 10 60125 (mean 35) mean 12 mean 5 12510 to 6 125180 (mean 90)

SVC =superior vena cava; IYC =Inferior vena cava; RA =right atrium; RV = right ventricle; PA = pulmonary artery; PCW = pulmonary capillary wedge ; LA =left atrium; LV=left ventricle.

in the absence of an ASD and even if only one pulmonary vein drains anomalously.U As pulmonary vascular resistance and pulmonary artery pressure rise, right heart failure develops. Because of the directcommunication between the pulmonary veins and the right atrium or systemic veins, right heart failure results in pulmonary edema. In the patient with concomitant lung disease, such as the one presented here, pulmonary hypertension may be the result oflong-standing APVC, lung disease, or both. In any case, pulmonary edema causes further cardiopulmonary compromise. Vasodilator drugs are useful in treating left ventricular failure. Their effectiveness in the treatment of pulmonary hypertension is more limited.' They may act directly or indirectly on arteries, veins or both, and may act preferentially on pulmonary or systemic vessels. For treating APVC with pulmonary hypertension, right ventricular failure and pulmonary edema, one might look for an agent with the following effects: 1) reduction in pulmonary vascular resistance, 2) increase in venous capacitance, 3) little or no reduction of systemic vascular resistance, and 4) no myoeardial depression. Net effect would be improvement in right ventricular function and pulmonary blood flow, relief of pulmonary congestion, and maintenance of systemic blood pressure. Nitrate therapy causes dilatation of pulmonary arteries and veins and systemic venous capacitance vessels at doses that have minimal effect on systemic arterial resistance vessels.' Nitrates are not cardiac depressants. Pearl and coworkers' have shown beneflcial hemodynamic effects of nitrate therapy in patients with chronic pulmonary hypertension. However, in some patients with pulmonary hypertension, the venodilating effect of nitrates may be detrimental.' If pulmonary vascular resistance fails to fall sufficiently, the decrease in venous return to the heart may cause a fall in cardiac output. In APVC, where pulmonary blood is returning to the systemic veins or right atrium, the fall in venous pressures (both systemic and pulmonary) induced by nitrate therapy might facilitate transpulmonary blood flow, increase both right and left ventricular output, and decrease pulmonary congestion. These beneflcial effects might occur even in the absence of a pulmonary arterial vasculature responsive to treatment with vasodilators. In other disease states associated with pulmonary hypertension, there is evidence that the pulmonary venodilating action of nitrates may playa key role in decreasing pulmonary vascular resistance.' The fact that our patient's pulmonary capillary wedge pressure was higher than either her left or right atrial pressures, in the absence of any angiographically demonstrable obstruction to pulmonary venous return, suggests that pulmonary venous tone may have been signiflcant. Our patient had moderate pulmonary hypertension and right heart failure manifested by pulmonary edema. Oxygen and diuretics alone were ineffective treatment, and hydralazine therapy caused hypotension. Administration of nitroglycerin caused prompt and marked improvement in her pulmonary edema, and its discontinuation led to immediate deterioration. The development of new noninvasive diagnostic methods, such as digital subtraction angiography,• computerized tomography," and magnetic resonance imaging, U may lead to an increased recognition of adults with APVC. Our experience suggests that nitrates may be beneflcial in the preoperative or nonsurgical management of CHEST I 89 I 5 I MAY, 1988

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selected patients with APVC . Early surgical correction of partial APVC may be warranted, even in the absence oflarge shunts. Not only can pulmonary vascular obstructive disease be a late complication of APVC, but the development oflung disease can cause partial APVC to gain physiologic significance. REFERENCES 1 Healey JE . An anatomic survey of anomalous pulmonary veins: their clinical significance. J Thorac Surg 1952; 23:433-44 Dale G. Pulmonary tuberculosis associ2 Conant JS, Kurland ated with anomalous common left pulmonary vein entering the left innominate vein. J Thorac Surg 1947; 16:422-26 3 Sepulveda G, Lukas OS, Steinberg I. Anomalous drainage of pulmonarY veins: clinical, physiologic and angiocardiographic features . Am J Med 1955; 18:883-99 4 Saalouke MG, Shapiro SR, Perry L\v, Scott LP. Isolated partial anomalous pulmonary venous drainage associated with pulmonary vascular obstructive disease . Am J Cardioll977; 39:439-44 5 Babb JD, McGlynn TJ, Pierce WS, Kirkman PM. Isolated partial anomalous venous connection: a congenital defect with late and serious complications. Ann Thorac Surg 1981; 31:540-43 6 Packer M, Halpern JL, Brooks KM, Bothlauf EB, Lee WH o Nitroglycerin therapy in the management of pulmonary hypertensive disorders. Am J Med 1984; 76(6A):67-75 7 Pearl RG, Rosenthal MH, Schroeder JS, Ashton CPr. Acute hemodynamic efFects of nitroglycerin in pulmonary hypertension. Ann Intern Med 1983; 99:9-13 8 Packer M. Vasodilator therapy for primary pulmonary hypertension. Ann Intern Med 1985; 103:258-70 9 Sider L, Fisher MR, Mintzer RA. The evaluation of partial anomalous pulmonary venous return with the use of digital subtraction angiography. Chest 1984; 86:97-99 10 Gross BH, Glazer GM, Kissner DG . cr of unusual mediastinal vascular abnormalities. Computerized Radioll984; 8:369-74 11 Julsrud PRo Ehman RL. The "broken ring" sign in magnetic resonance Imaging of partial anomalous pulmonary venous connection to the superior vena cava. Mayo Clin Proc 1985; 60:

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Surgical Implications of Intraoperative Postlnfarctlon ventricular Rupture* Frwd H. Edwards, M.D.; Robert A Albus, M.D.; G. M. Graeber, M.D., EG .G.P.; and George A Besch, M.D.

Rupture of the left ventricular free wall is a common cause ofdeath in the first several days following acute myoeardial infarction (MI). This catastrophic event is usually fatal, but there are at least 23 cases in the English-language literature in which prompt surgical intervention has been successful. 1.. We describe a patient whose left ventricular free wall rupture sealed by clot for several hours, only to spontaneously rupture the ventricle once again during the course of surgical exploration. CASE REPORT A 55-year-old man with no previous cardiac complaints was admitted with substernal chest pain. Cardiac enzymes and electrocardiographic findings were both consistent with an acute anteroseptal MI and the patient was treated accordingly. He was making an uneventful recovery until the fifth day after infarction, when he collapsed. He was monitored by telemetry and no dysrhythmias had been noted. Systolic blood pressure was 80 mm Hg with a sinus tachycardia of 120 beatslmin. A 15 mm Hg pulsus paradoxus was present, and he had moderately distended neck veins. There were no new findings on cardiac auscultation. Electrocardiographic and chest x-ray examination results were unchanged from previous studies. Pulmonary artery catheterization showed that the central venous pressure and the pulmonary capillary wedge pressure were both 16 mm Hg. Echocardiographic examination demonstrated pericardial fluid collection. The patient was urgently explored through median sternotomy. Upon opening the pericardium, a considerable amount of clotted blood was found, but there was no active bleeding. The clot was removed and an area of infarction with subepicardial hemorrhage was found between the anterior descending coronary artery and the first diagonal branch. Even though the area of infarction and hemorrhage was apparent, no site of cardiac rupture could be identified (Fig 1). Just as closure was begun, however, an Infarcted area of the anterior left ventricular free wall spontaneously ruptured approximately 3 cm above the apex (Fig 2). The bleeding was quickly controlled and cardiopulmonary bypass was instituted. The defect was plicated with multiple large pledgets and an overlying Teflon patch that affurded excellent hemostasis (Fig 3). Attempts were made to preserve the distal anterior descending coronary artery, but this proved technically impossible. In order to attain a secure closure of the cardiac rupture, this vessel and the second diagonal branch had to be incorporated into the repair and were unavoidably obliterated. An intra-aortic balloon pump was placed and the patient was slowly weaned from bypass. The intra-aortic balloon pump was removed six days after surgery

'Ibis case report documents that venbicular myocardial rupture after acute infarction may seal with dot, only to rupture again with potentially lethal consequences. At exploration, the dot over a fresh rupture was intact. As dosure was started, the dot disrupted, causing severe hemorrhage. Rupture was successfully repaired and the patient survived.

*From the Department of Cardiothoraclc Surgery and Cardiology Services, Walter Reed Army Medical Center, Division of Surgeryl Walter Reed Army Institute of Research, Washington, D.C., ana the Uniformed Services University of the Health Sciences, F. Edward Hebert School of Medicine, Bethesda. The opinions and assertions contained herein are the private views of the authors and are not to be construed as official or as reflecting the views of the Department of the Army or the Department of Defense. Reprint I"8quuts: Dr: Graeber; Walter Reed Army Medical Genter, WtUhlngton, DC 20307

Frcuas 1. Intraoperative appearance of the heart after removal of the overlying clot. The discolored, hemorrhagic areas along the anterior surface represent infarcted myocardium . The base of the heart lies to the left. Intraoperative Postinlarctlon ventricular Rupture(Edwards et aI)