Aneurysm of sinus of Valsalva

A 14-year-old adolescent girl presented with severe congestive heart failure, progressive throughout 3 months. A precordial thrill, machinery-like murmur, and right bundle branch block were noted. Death occurred despite digitalis and diuretic therapy and removal of pleural and ascitic fluid. The autopsy revealed 2 multilocular cystic structures in the interventricular septum consistent with being spontaneously drained valve ring abscesses. One of these lesions formed a fistulous communication that penetrated through the interventricular septum between the right aortic sinus of Valsalva and the crista supraventricularis that connected to the right ventricle. Another lesion, an adjacent separate but similar cystlike structure, communicated only with the left ventricular cavity. Although the cause of these lesions is uncertain, it seems probable that they are the residue of spontaneously drained and healed valve ring abscesses. Max Brödel, a medical illustrator and the first director of the Department of Art as Applied to Medicine at The Johns Hopkins University, drew previously unpublished figures of this patient's cardiac lesions. These illustrations exhibit Brödel's superb command of both art and medicine essential to his ability to make complex anatomic relationships demonstrable. We discuss Brödel's career and his influence on both the art and science of medicine.

(Arch Pathol Lab Med. 2005;129:1155-1158)

THE CASE

In July 1937, a 14-year-old adolescent girl was admitted to The Johns Hopkins Hospital with severe congestive heart failure. Her symptoms began 3 months before admission with dyspnea on exertion and fatigue. Her face also appeared "puffy." Before symptom onset, the mother stated that the patient's health was unremarkable. Since 6 weeks before admission, a gradual increase in the size of the abdomen and pronounced lower-extremity edema were noted. By the time of admission, the patient's dyspnea had progressed until it was extreme even at rest.

Her physical examination demonstrated marked edema of both the face with eyelid closure and the abdominal wall, as well as bilateral rales. The heart was greatly enlarged, and a loud to-and-fro murmur was audible in all auscultated areas. An intense continuous thrill was palpable best midway between the sternum and the apical pulse at the fourth intercostal space. At this point, the murmur was also loudest. The results of laboratory studies for white and red blood cell counts and total protein were normal. A total of 600 and 1000 mL of sterile transudative fluid were removed by thoracentesis and paracentesis, respectively.

The patient's hospital course was characterized by progressive decline. She was placed in an oxygen tent. Her temperature dropped below normal, and her pulse remained rapid. Digitalis was administered for the first 3 days and then diuretics were given but were ineffectual. An electrocardiogram performed on the fifth hospital day showed sinus tachycardia, first-degree heart block, and right bundle branch block. These abnormalities were attributed to digitalis toxicity, and a subsequent electrocardiogram showed similar but improved findings after discontinuing use of this medication. On the seventh hospital day, the patient's condition was dire, she deteriorated further, and then she died.

THE AUTOPSY REPORT

At autopsy, the body, 152 cm long and weighing 52 kg, showed marked anasarca with more than 1 L of ascitic fluid and more than 500 mL of serous fluid in each pleural cavity. There were healing granulomas and caseous foci, presumably tuberculosis, in mesenteric and peripancreatic lymph nodes. No other evidence of an infectious process was found. All organs other than the heart demonstrated the severe effects of chronic passive congestion without any evidence of low cardiac output.

The heart, however, was enlarged and weighed 500 g. The right atrium and ventricle were severely dilated and hypertrophied. The left-sided chambers were less so. The chordae tendinea of the septal leaflet of the tricuspid valve were bound to a pearly white fibrous endocardial plaque on the crista supraventricularis, probably producing tricuspid insufficiency. Within this plaque, a defect approximately 5 mm in diameter entered an irregular saccular cavity, which communicated via a second 5-mm-diameter orifice with the right aortic sinus of Valsalva just below the right coronary artery ostium (Figure 1). The lining of the irregular intracristal and intraseptal multiloculated cavity showed a layer of dense organized connective tissue. On its luminal side, small amounts of recent thrombus were present focally. Its adventitial side had collections of chronic inflammatory cells that were also found in the adjacent myocardium. A second separate irregular sacculated pouch was located anterior to the first in the septum and anterior left ventricular wall. It communicated with the left ventricle through an orifice surrounded by pearly endocardial plaque.

DISCUSSION OF THE CASE

The patient's clinical course was ascribed to the profound right-sided congestive heart failure, and the later, milder left-sided congestive heart failure produced by the aorta-to-right ventricle communication of the sinus of VaIsalva aneurysm. Rupture of the aneurysm into the right ventricle probably occurred approximately 3 months before death, but how long it had been progressing before that point is not known.

Congestive heart failure has multiple causes in the pediatrie population. Congenital heart defects, cardiomyopathies, and cardiac dysfunction as a result of repair of a heart abnormality are the primary causes of congestive heart failure in children.1 By history and autopsy pathology, these etiologies may be ruled out as less likely causes of this patient's cardiac lesions.

Aortic sinus of Valsalva aneurysms have been shown to demonstrate primary right-sided valvular heart disease,2 with heart failure the result of the simulated valvular disease. The cause of these lesions may not be clear, even in autopsy studies,2 and is not clear in this patient. Syphilis, formerly a frequent cause of sinus of Valsalva aneurysms, was not present by either clinical or pathologic criteria. A healing tuberculosis primary complex was found in the mesenteric nodes of the patient. If the cardiac lesions had been produced by tuberculosis, then there should have been evidence of systemic dissemination. Without this evidence, tuberculosis as a cause of the patient's condition is a highly unlikely circumstance. It is difficult to conceive of a developmental abnormality that could produce 2 such multiloculated cystic structures in the ventricular musculature. The location of the left-sided ostia of the lesions would be unusual for diverticula.

The autopsy revealed 2 multilocular cystic structures in the interventricular septum of the patient's heart. The pathogenetic mechanism favored to explain the lesions is that they may be the late result of an infection, possibly spontaneously drained and healed valve ring abscesses. Ruptured valvular abscesses have been reported in the literature in the setting of subacute bacterial endocarditis,3 mitral valve replacement for bacterial endocarditis,4 and streptococcal endocarditis due to streptococcal pneumonia.5 Such lesions may be multiple and commonly perforate onto an endocardial surface. Subsequent enlargement of the lesions and the perforation of one into the right ventricular cavity could be caused by the action of the blood pressure within the aneurysm.

Clinical diagnosis of sinus of Valsalva aneurysms may be difficult even after rupture, as occurred in the present case. Contemporary noninvasive diagnostic procedures, such as echocardiography, may facilitate diagnosis. Transesophageal echocardiography has been shown to be superior to transthoracic echocardiography in the detection of cardiac lesions such as endocarditis and valve ring abscesses.6 However, echocardiography may miss a valve ring abscess7; therefore, a high index of suspicion is still required. The presence of a continuous murmur may be a helpful clue to aneurysm rupture. Surgery has proved to be effective in the treatment of unruptured8 and ruptured9 sinus of Valsalva aneurysms.

MAX BRÖDEL

Max Brödel (1870-1941) was an important figure in the art of medical illustration. He was born in Leipzig, Germany, on June 8, 1870.10 A talented artist, he enrolled in the Academy of Fine Arts in Leipzig after leaving high school. While a student at the Academy, Dr Carl Ludwig (1816-1895), the famous director of the Institute of Physiology and professor of physiology at the University of Leipzig, contacted the academy to request recommendations for a substitute medical illustrator. Brödel's name was put forward by the school's director, and he began work in Ludwig's laboratory in the summer of 1888.

Brödel stayed 18 months, working on anatomic and histologie drawings. His first assignment for Ludwig was a colored drawing of a microscopy slide through the brain cortex magnified 150 times on a 6-in-wide and 1-yd-long illustration.10 This and other experiences with Dr Ludwig fashioned Brödel's future artistic and scientific capabilities.

The international renown of Carl Ludwig attracted numerous young physicians and scientists from abroad. Two whom Brödel met were future luminaries of The Johns Hopkins University, William H. Welch (1850-1934) and Franklin P. Mall (1862-1917). Mall, the future head of anatomy at Johns Hopkins, was especially influential in attracting Brödel to the United States in 1894.

Initially at Johns Hopkins, Brödel worked with Howard A. Kelly (1858-1943), who was writing his textbook, Operative Gynecology," which would establish Kelly as the leading gynecologist in the United States. Brödel achieved remarkable success with Kelly, both as an artist and as a scientist.12 He contributed chapters on anatomy to textbooks by Kelly" and Thomas Cullen (1868-1953)." He also made several medical discoveries in his own right, such as dissection studies of the intrinsic blood vessels of the kidney defining an avascular line for nephrectomy (Brödel's white line) and the description of a modified suture technique for nephroplexy (the Brödel suture).14

Within his own specialty of medical illustration, Brödel also made contributions. He developed a drawing method using carbon dust and a stipple board technique. A special paper coated with chalk or china clay with a stipple pattern allowed Brödel to render living tissue in lifelike quality using a carbon pencil and dust to produce halftone effects.10

Throughout his long career at Johns Hopkins, Brödel achieved several other notable successes. The Department of Art as Applied to Medicine was established at The Johns Hopkins University in 1911. At the time, the Mayo Clinic in Rochester, Minn, was attempting to recruit Brödel. Established in recognition of Brödel's importance and influence as an illustrator and to keep him in Baltimore, the new department was the first of its kind in the world to train students specifically as medical artists.10

Brödel left an impressive legacy of artistic and scientific achievement. His technique of completely mastering a topic before drawing it allowed for this high level of demonstrative skill:

I know that it would be useless for me to compete with the camera in the realistic or imitative field. It was necessary to originate a different type of picture, one that would show far more than any photograph could ever do. To make such a picture is much more difficult. The artist must first fully comprehend the subject matter from every standpoint: anatomical, topographical, histological, pathological, medical, and surgical. From this accumulated knowledge grows a mental picture, from which again crystallizes the plan of future drawing. A clear and vivid mental picture must always precede the actual picture on paper. The planning of the picture, therefore, is the all-important thing, not the execution.10

Brödel made previously unpublished illustrations of the heart of the 14-year-old girl who died in 1937 after 3 months of progressive heart failure (Figures 2 and 3). Brodel's drive to create "a different type of picture" than a photograph is shown here in the contrasting pathologic photograph (Figure 1) and Brödel's drawings (Figures 2 and 3). The illustrations exhibit Brödel's superb command of both art and medicine essential to his ability to convey complex anatomic relationships.

This work was supported by The Department of Art as Applied to Medicine at The Johns Hopkins University School of Medicine, which provided the original art by Max Brödel.

References

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4. Weale AE, Mutter J, Collins CM. Rupture of a ventricular abscess after mitral valve replacement. Br Heart J. 1992;67:271-272.

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10. Crosby RW, Cody J. Max Brödel: The Man Who Put Art Into Medicine. New York, NY: Springer-Verlag lnc; 1991.

11. Kelly HA. Operative Cynecology. New York, NY: Appleton; 1898.

12. Schultheiss D, Jonas U. Max Brodel (1870-1941) and Howard A. Kelly (1858-1943): urogynecology and the birth of modern medical illustration. EurJ . Obstct Gynecol Reprod Biol. 1999;86:113-11 5.

13. Cullen TS. Embryology, Anatomy, and Diseases of the Umbilicus Together With Diseases of the Urachus. Brodel M, illus. Philadelphia, Pa: WB Saunders Co; 1916.

14. Schultheiss D, Engel RM, Crosby RW, Lees GP, Truss MC, Jonas U. Max Brödel (1870-1941 ) and medical illustration in urology. I Urol. 2000;164:1137-1142.

Brendan P. Lucey, MD; Caroline Bedell Thomas, MD[dagger]; Crover M. Hutchins, MD

Accepted for publication May 3, 2005.

From the Department of Neurology, Washington University Medical Center, St Louis, Mo (Dr Lucey); and Department of Pathology, The Johns Hopkins Medical Institutions, Baltimore, Md (Dr Hutchins).

The authors have no relevant financial interest in the products or companies described in this article.

[dagger] Dr Thomas is deceased but contributed significantly to the manuscript before her death.

Reprints: Brendan P. Lucey, MD, Department of Neurology, Washington University Medical Center, Campus Box 8111, 660 S Euclid Ave, St Louis, MO 63110 (e-mail: brendanlucey@hotmail.com).

Copyright College of American Pathologists Sep 2005
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