DISCUSSION
Koerner and Sun 2 reported three cases of mediastinal lipomatosis following large doses of systemic corticosteroid therapy. Thereafter, several authors 1 ,3-6 reported a number of similar cases, All reported cases had in common some evidence of clinical Cushing's syndrome (either primary or iatrogenic) . In simple obesity, excessive fat is generally stored at various body sites, notably in the subcutaneous tissue, omentum, mesentery, and perirenal tissue. Although more fatty tissue may be present within the mediastinum in obese persons, an amount of fat sufficient to produce significant mediastinal widening on the chest roentgenogram is rare, In mediastinal lipomatosis the mediastinal widening is bilateral. The contour is smooth and sharply defined. The density of the abnormal shadow is not as pronounced as other masses or surrounding structures. Although fatty tissue is radiolucent, its lucency is lost to a certain degree because of adjacent, more lucent pulmonary tissue. 4 The absence of tracheal compression or narrowing is significant, since firm or encapsulated lesions may cause a pressure effect on the trachea. The absence of a definable mass in the lateral view is a significant finding, suggestive of lipomatosis. The presence of a pericardiaI fat pad is another helpful sign of the fatty nature of the mediastinal widening. The radiologic features described herein are identical to mediastinallipomatosis secondary to systemic steroid therapy or primary Cushing's syndrome. The differential diagnosis of symmetric widening of the superior mediastinum in adults is limited. In acute mediastinitis, mediastinal hemorrhage, and dissecting aneurysm, the diagnosis is generally made without much difficulty on the basis of a correlation of radiographic findings with the clinical history. REFERENCES
1 Price JE, Rigler LG: Widening of the mediastinum resulting from fat accumulation. Radiology 96:497-500, 1970 2 Koerner HJ, Sun Di-C: Mediastinal lipomatosis secondary to steroid therapy. Am J RoentgenoI98:461-464, 1966 3 Bodman SF, Condemi JJ: Mediastinal widening in iatrogenic Cushing's syndrome. Ann Intern Med 67:399-403, 1967 4 Santini LC, Williams JL: Mediastinal widening in Cushing's syndrome. N Engl J Med 284:1357-1358,1971 5 Teates CD: Steroid-induced mediastinal lipomatosis. Radiology 96:501-502, 1970 6 Shuman BM: Mediastinal lipomatosis complicating steroid thpr~~" - r regional enteritis. Gastroenterology 61 :244-246,
, AUGUST, 1976
Ruptured Aneurysm of Aortic Sinus of Valsalva into Right Atrium* Associated Atrioventricular Block Presumably Caused by Aneurysmal Compression of His Bundle Nobuyuki Anzai, M.D.; Tadahiko Okada, M.D.; roshinori Takanashi, M.D.; Akira Sano, M.D.; and Manabu ramada, M.D.
We have seen a case of rupture of an aneurysm of the noncoronary sinus into the right atrium. Surgery revealed an aneurysmal mass the size of the tip of an index finger extending through the inter-atrial septum down to just above the tricuspid valvular ostium. An electrocardiogram showed first-degree atrioventricular block, while the His bundle electrogram demonstrated the presence of disturbances in the intra-atrial as well as His bundle conduction. The disturbance in His bundle conduction was interpreted as being due to compression of the His bundle by the aneurysm. of an aneurysm of the sinus of Valsalva, with Rupture consequent abrupt hemodynamic changes, entails a
heavy cardiac load and progressive cardiac failure, Hence, the prognosis is always grave. In this paper, we present a case of rupture of an aneurysm of the sinus of Valsalva in which atrioventricular block developed, presumably due to compression of the conducting system by the aneurysm. CASE REPORT
Our patient was a 39-year-old man. When he was a child, he was said to have "heart disease," hut the details are not known. The patient had been in good health until 1969, when he consulted a physician for a common cold and was found to have heart murmurs. Towards September 1973, he developed palpitation and dyspnea during exercise, and his condition gradually worsened thereafter. Precordial pressure, orthopnea, coughing, and expectoration supervened, and a feeling of abdominal fullness developed later. With these symptoms the patient was admitted to Higashi Nagano National Hospital, Nagano, Japan, on Oct 1,1973. On admission the patient's height was 171 cm (5 ft 7 in), and his body weight was 59 kg (130 Ib). His pulse rate was 102 beats per minute and regular, with the presence of socalled Corrigan's pulse. The jugular vein was slightly engorged. The liver was palpable two 6ngerbreadths below the costal margin, but the spleen was not felt. There was slight edema of the lower extremities. On auscultation, grade 3/6 toand-fro radiating murmurs, loudest over the lower end of the sternum, were heard. At the apex, systolic murmurs of grade 3/6 were audible. The second pulmonic heart sound was not accentuated. No abnormalities were noted on blood examination, urinalysis, or tests of hepatic and renal function. The chest x°From the Department of Cardiology and Cardiac Surgery, Higashi Nagano National Hospital, Nagano, Japan. Reprint requests: Dr. Anzai, 417 Uwano, Nagano, Nagano Prefecture, Javan
RUPTURED ANEURYSM OF AORTIC SINUS OF VALSALVA 309
~l '~
II
111
~i
Table I-Data /rom Intracardiac Catheterisation *
Position
z
V
Maximum/Minimum Pressure, mm Hg OO
Oxygen Saturation, percent
•_ _c __ - - - - - - l
I
Superior vena cava
22/8
(13)
61.9
~'
Inferior vena cava
15/9
(13)
76.5
Right atrium Upper Middle Lower
22/4 22/6 18/8
(14) (13) (13)
76.5 88.8 83.4
Right ventricle Outflow Apex Inflow
60/-5 (35) 70/10 (30) 65/0 (30)
91.3 95.3 87.5
-t.:-_._ .. --,
4
~-
_
_. ~'
-.---.-----. . - - . - ._ . ... _ .. .'
ITLU
AVF
FiGURE 1. Preoperative ECG demonstrating left ventricular hypertrophy and first degree atrioventricular block. ray film showed marked pulmonary congestion and enlargement of the cardiac silhouette to both sides, with a cardiothoracic ratio of 69 percent. The electrocardiographic findings revealed the following: regular sinus rhythm; P-R interval, 0.28 second; QRS axis, +60°; P wave slightly heightened in leads 2, 3, aVF, and VI and diphasic in lead VI; deep S wave in leads V3 and V4; P wave taller before surgery than after surgery; and evidence of left ventricular hypertrophy (Fig 1). The findings from the His bundle electrogranl included the following: P-A interval, 76 msec; atrio-His interval, 20 msec; His-ventricle interval, 84 msec; and H interval, 27 msec (Fig 2). Right cardiac catheterization disclosed enlargement of the right atrial and right ventricular cavities, and it was difficult to insert a catheter into the pulmonary artery. Right atrial, superior vena caval, inferior vena caval, and right ventricular pressures were all elevated. Oxygen saturation was found to be increased in the right atrium and even more so in the right ventricle (Table 1). The left-to-right shunt ratio was 78 percent. During left cardiac catheterization a catheter was easily advanced from the aorta into the right atrium. The aortic pressure was 130/50 mm Hg, the left ventricular pressure was 130/0 mm Hg, the left ventricular end-diastolic pressure was 16 mm Hg, and the A-wave pressure was 24 mm Hg (Table 1). Retrograde aortographic studies revealed regurgitation of the contrast agent from the noncoronary sinus into the right atrium, with resultant visualization of the right
Aorta
130/50 (70)
Left ventricle
130/0
Left ventricle, end of diastole
(42)
16
°Data demonstrate oxygen step-up at right atrium and double oxygen step-up at right ventricle. ooNumbers within parentheses are means. atrium (Fig 3). The aforementioned findings established the diagnosis of rupture of an aneurysm of the sinus of Valsalva into the right atrium. In view of the presence of progressive heart failure, surgery was performed on Nov 8, 1973. The heart was exposed by median sternotomy. The right atrium and ventricle both appeared to be markedly enlarged, and there was a thrill felt most strongly over the lower portion of the right atrium. With the aid of extracorporeaI circulation, a venting tube was inserted through the left ventricular apex, the aorta
1 sec
FIGURE 2. His bundle electrogram demonstrating prolongation of P-A and His-ventricle intervals; H wave had two peaks and was somewhat prolonged.
310 ANZAI ET AL
FiGURE 3. Retrograde aortogram demonstrating of aneurysm and right atrium (frontal view).
visualizatio~
CHEST, 70: 2, AUGUST,
197~
was cross-clamped when the venous temperature reached 30°C (87°F), and the right atrium was incised under anoxic arrest. An expansile mass the size of the tip of an index finger was seen near the annulus of the tricuspid valve, extending from the atrial septum toward the posterior leaflet of the tricuspid valve. There were two perforations at the top of the mass, where it was partially adherent to the atrial septum. The mass could easily be separated from the right atrial wall with a pickup. A slight indentation of the right atrial wall was left after the separation. The mass was incised at the base. This resulted in a defect of the sinus wall approximately 1 cm in diameter. The opening was closed with a patch suture, and further sutures were applied to ensure the closure. The immediate postoperative course was uneventful, without any pronounced cardiac failure or drop of blood pressure; however, transient second-degree atrioventricular block, supraventricular premature beats, and atrioventricular junctional rhythm were observed on the ECG. The P-R interval remained the same as it was before surgery. A postoperative x-ray 6lm revealed a reduction in the size of the cardiac shadow without evidence of a relapse of the aneurysm. DISCUSSION
It is believed that electrocardiographic findings generally are of little value in the diagnosis of rupture of an aneurysm of the sinus of Valsalva; however, it has been pointed out that rupture into the right atrium may be associated with prolongation of the P-R interval, incomplete right bundle-branch block, and atrioventricular block. 1 - 4 Furthennore, electrocardiographic evidence of sudden right atrial overload, such as atrioventricular nodal rhythm, atrial fibrillation, atrial tachycardia, and atrial premature beats, has also been reported. 1,s-7 In the present case a prolonged P-R interval was noted on the ECG and prolongation of the P-A and Hisventricle intervals on the His bundle electrogram, with the H wave being diphasic and slightly prolonged. The atrio-His interval was considered to be at the upper limit of the nonnal range. 8 There was disturbance of intraatrial conduction, although it was uncertain whether the prolongation of the P-A interval was due to mere right atrial overload or to disturbance of the internodal tract resulting from the aneurysm. Because of the absence of bundle-branch block, the prolongation of the His-ventricle interval was very likely due to a lesion in the lower portion of the His bundle above the bifurcation. Moreover, reported evidence indicates that the H wave may be split into two spikes (H and H') in the presence of a lesion of the His bundle. 9 In the present case the H wave had two peaks and was somewhat prolonged, suggesting the presence of ~ conduction disturbance of the His bundle. At the time of surgery, the tip of the aneurysm ·was found to be located close to the tricuspid valvular orifice and adhering to the interatrial septum. This finding strongly suggests the possibility that the aneurysm was compressing the His bundle. Micks 4 and Duras 3 reported cases in which compression of the His bundle by an aneurysm caused complete atrioventricular block. In the cases reported by Herson and Symons,10 compression of the atrioventricular node by the aneurysm resulted in atrioventricular nodal rhythm. Fur-
CHEST, 70: 2, AUGUST, 1976
thermore, Hall and Pickard 7 reported a case in which first-degree atrioventricular block with progressive prolongation of the P-R interval was observed ante mortem and in which autopsy findings strongly suggested the relationship between the presence of an aneurysm and the occurrence of atrioventricular block. From the aforementioned, it seems warranted to interpret the prolongation of the His-ventricle interval observed in the present case as having resulted from compression of the His bundle by the aneurysm. The patient is currently under close observation, considering the likelihood of his developing complete atrioventricular block in the future. Surgery in the present case did not result in a significant shortening of the P-R interval, suggesting a permanent organic change in the conducting system. A careful follow-up of the patient with a repeat His bundle electrogram seems to be indicated.
1 Oram S, East T: Rupture of aortic sinus of Valsalva into right side of the heart. Br Heart J 17 :541, 1955 2 Hemnann GH, Schofield NP: The syndrome of rupture of aortic root or sinus of Valsalva aneurysm into right atrium. Am Heart J 34:87, 1947 3 Duras RH: Heart block with aneurysm of the aortic sinus. Br Heart J 2:63, 1956 4 Micks RH: Congenital aneurysms of all three sinuses of Valsalva. Br Heart J 2:63, 1940 5 Macleod A: Cardio-aortic fistula. Br Heart J 6: 194, 1944 6 Gerbode FG, Osborn JF, Johnston JB, et al: Ruptured aneurysm of the aortic sinuses of Valsalva. Am J Surg 102:268, 1961 7 Hall B, Pickard SD: Unsuspected rupture of aortic sinus of aneurysm into right atrium. Am J Cardiol 3:404, 1959 8 Narula OS: Conduction disorders in the A-V transmission system. In Cardiac Arrhythmias. New York, Grune and Stratton Inc, 1973, p 259 9 Narula OS, Scherlag BJ, Samet P, et al: Atrioventricular block: Localization and classification by His bundle recordings. Am J Med 50: 146, 1971 10 Herson RN, Symons M: Ruptured congenital aneurysm of the posterior sinus of Valsalva. Br Heart J 8: 125, 1949
Closure of a Ventricular Septal Defect through the Aortic Valve* A Note of Caution Regarding Resultant Narrowing of the Aortic Valve Annulus Scott Stewart, M.D.
Closure of ventricular septal defects through the aortic valve annulus has been described for a variety of cardiac anomalies. This technique is most appropriate when o From
the Department of Surgery, Division of Cardiothoracic Surgery, University of Rochester, Rochester, NY. Reprint requests: Dr. Stewart,601 Elmwood Avenue, Rochester, New YOf'k 14642
CLOSURE OF VSO THROUGH AORTIC VALVE 311