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P&S Medical Review: Apr 1995, Vol.2, No.2
Clinicopathological Conference : A 48- year- old Woman with a Known Cardiac Disease Presents in Extremis
Robert H. Heissenbuttel, M.D., Giorgio Cattoretti, M.D.,
John H. M. Austin, M.D., and Abbie I. Knowlton, M.D.
Columbia University College of Physicians and Surgeons, New York, NY
Presentation of Case
This 48-year-old Hispanic woman with known cardiac disease was brought to the Columbia-Presbyterian Medical Center's Emergency Department by EMS, unresponsive and with no measurable blood pressure.
The patient had a history of hypertension and cigarette smoking, but was in generally good health until fifteen months prior to her death, when she developed episodes of chest tightness and dyspnea on exertion, accompanied by diaphoresis, nausea, and an urge to urinate and defecate. Over the next several months, her exercise tolerance gradually decreased to half a block. She developed four-pillow orthopnea and occasional pedal edema, but denied paroxysmal nocturnal dyspnea. She became increasingly housebound.
Eleven months prior to death, she was hospitalized after a thirty-minute episode of chest pain, dyspnea, and loss of bowel and bladder control. She was afebrile, with a blood pressure of 150/84 mm Hg, pulse 80, and respirations 18. On physical exam, there was no elevated jugular venous pressure and the PMI was not laterally displaced. She had a 1/6 holosystolic ejection murmur and bibasilar rales. She had no hepatomegaly, but had a hernial mass at the site of a scar in the left lower quadrant. There was mild peripheral edema.
The patient's chest pain resolved with sublingual nitroglycerin, and a myocardial infarction was ruled out by electrocardiogram and cardiac enzymes. A subsequent echocardiogram revealed a thickened mitral valve with severe regurgitation, normal ventricular wall motion, and an ejection fraction of 60 percent. She was discharged on enalapril, metoprolol, and a daily two percent nitroglycerin ointment patch, and scheduled for cardiac catheterization in two months.
The cardiac catheterization, nine months prior to death, showed non-obstructing coronary artery disease, normal left ventricular function, an ejection fraction of 71 percent, and severe mitral insufficiency. She was admitted and underwent open heart surgery for a mitral valve repair with a Duran Ring. The post-operative echocardiogram revealed only trace mitral regurgitation. She was begun on prophylactic anticoagulation with coumadin, which was continued for six months.
One month prior to her death, she was hospitalized for severe dyspnea. She was afebrile, with a blood pressure of 110/70 mm Hg, pulse 90, respirations 24, and weight 70.5 kilograms. She was pale and in mild respiratory distress. There was 10 cm of jugular venous distention at 45 degrees. There was no lymphadenopathy. Occasional inspiratory wheezes were heard at the lung bases. The heart rate was regular, and the PMI was not laterally displaced. A 1/6 systolic murmur was noted along the left sternal border; no gallops were appreciated. Her abdomen was softly distended and slightly tender to palpation. There was pitting edema to the thighs. She was started on furosemide 40 mg BID. Laboratories on admission are shown in Tables 1 - 2.
| Variable | Value |
| White-Cell count (per mm 3 | 11800 |
| Differential count (percent) | |
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| Hemoglobin (mg/dL) | 9.7 |
| Hematocrit (percent) | 30 |
| Platelet count (per mm 3) | 452000 |
Her furosemide was increased to 60 mg BID, and she was begun on digitalis. Her dyspnea and peripheral edema improved after two weeks of therapy and she was discharged with a weight of 61.8 kg. She remained tender in the abdomen. Her medications upon discharge included digoxin (0.25 mg per day), KDur, ranitidine, and an antacid.
On the day prior to her death, the patient presented to the Emergency Department complaining of periumbilical pain and diarrhea. Her temperature was 101.2oF, and her blood pressure was 90/60 mm Hg. Urinalysis showed no protein, 5-7 white blood cells per high power field, and 2-3 red blood cells per high power field. The patient left without being examined.
| Variable | Value |
|---|---|
| Sodium (mEq/L) | 134 |
| Potassium (mEq/L) | 4.5 |
| Chloride (mEq/L) | 105 |
| Carbon dioxide content | 17 |
| Urea nitrogen (mg/dL) | 15 |
| Creatinine (mg/dL) | 1 |
| Glucose (mg/dL) | 60 |
| Calcium (mg/dL) | 8.5 |
| Magnesium (mEq/L) | 2.2 |
| Uric Acid (mg/dL) | 5 |
| Cholesterol (mg/dL) | 81 |
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| Total bilirubin (mg/dL) | 1.2 |
| Direct bilirubin (mg/dL) | 0.4 |
| Alkaline phosphatase (U/L) | 251 |
| Aspartate aminotransferase | 41 |
| Alanine aminotransferase | 22 |
| Lactate dehydrogenase | 337 |
The following day EMS was called; they found the patient unresponsive, with electrical activity by cardiac monitor but no measurable blood pressure. Advanced life support was initiated, and she was brought to the Emergency Department, arriving within 30 minutes. Arterial blood gases (ABG) on arrival were pH 6.97, pCO2 23 mm Hg, pO2 67 mm Hg, and bicarbonate 5 mEq/L. Laboratory tests on arrival (Table 3) were significant for a serum digoxin level of 3.0 ng/ml and a serum potassium of 6.9 mEq/L. Attempts at resuscitation were continued for another thirty minutes, to no avail. The patient was declared dead 37 minutes after arrival in the Emergency Department.
| Variable | Value |
|---|---|
| Sodium (mEq/L) | 133 |
| Potassium (mEq/L) | 6.9 |
| Chloride (mEq/L) | 90 |
| Carbon dioxide content (mEq/L) | 5 |
| Urea nitrogen (mg/dL) | 34 |
| Creatinine (mg/dL) | 1.7 |
| Calcium (mg/dL) | 9.5 |
| Uric Acid (mg/dL) | 10.5 |
| Cholesterol (mg/dL) | 70 |
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| Total bilirubin (mg/dL) | 3.5 |
| Direct bilirubin (mg/dL) | 1.9 |
| Alkaline phosphatase (U/L) | 298 |
| Aspartate aminotransferase (U/L) | 518 |
| Alanine aminotransferase (U/L) | 185 |
| Lactate dehydrogenase (U/L) | 1748 |
| Creatinine phosphokinase (U/L) | 249 |
| Serum Digoxin (ng/ml) | 3 |
Born in the Dominican Republic, the patient immigrated to the U.S. at age 40. She smoked one pack of cigarettes per day for many years and had a history of hypertension for which she received methyldopa; otherwise, she was in generally good health. Her past medical history was significant for a hysterectomy for fibroids at age 39, a subsequent incisional hernia, and an admission in 1990 for cellulitis of the hand secondary to a splinter. She also had a history of scoliosis and was diagnosed with spinal bifida at age 44. The patient's father is alive and well. Her mother died at age 49 of uterine cancer. She has eight siblings: seven alive and well, and one brother who died in infancy with "a leak in his spine."
Differential Diagnosis Dr. Knowlton: I selected a tough case. I thought that the terminal event was fairly straightforward, but that the underlying disease in this woman was puzzling, as indicated by the long history here. I am very eager to hear Dr. Heissenbuttel's presentation.
Dr. Heissenbuttel: A 48-year-old woman with hypertension was brought to the Emergency Department by EMS in extremis less than one year after mitral valve replacement. She could not be resuscitated and died less than two years after the onset of cardiac symptoms. Family, personal, and social history are non-contributory. She smoked one pack of cigarettes a day for 30 years. She had a history of hypertension which was well controlled, as far as we know.
In 1989, she was evaluated by neurology at CPMC for scoliosis and found to have spina bifida. Important at that time were her vital signs: blood pressure 130/70 mm Hg, and pulse 80 and regular. Her cardiac exam was normal, and her electrocardiogram was within normal limits.
Her next admission was in late 1990, approximately three years prior to death, when she was admitted with ascending lymphangitis involving the entire right arm secondary to an infected splinter. Her blood cultures were negative, and she was treated with intravenous antibiotics. Her temperature and white count returned to normal, and she was discharged after resolution of this infection. At the time, her cardiac exam was again normal-no murmur. Her electrocardiogram and chest X-ray were normal.
We did not hear from her again until early 1993. She was post-menopausal at that time, having had a bilateral salpingoophorectomy with her hysterectomy. She was hospitalized at the Allen Pavilion because of three months of recurrent episodes of chest pain, chest tightness, and dyspnea on exertion and at rest. These episodes were accompanied by sweating, nausea, and an urge to urinate and defecate-the latter two which are difficult to correlate with her cardiac symptoms. Her symptoms were progressive; she became housebound as she could only walk half a block. She developed four-pillow orthopnea and occasional episodes of ankle edema. She was admitted with the diagnosis of unstable angina and to rule out a myocardial infarction. Her blood pressure was 150/70 mm Hg, pulse 84 and regular, respirations 18 and non-labored. Jugular venous pressure was normal. Her lungs had bibasilar moist rales. She had a grade 1/6 holosystolic murmur at the cardiac apex, but no S3 or S4 gallop. Interestingly, in the many subsequent daily exams during that admission, no one described a heart murmur. Her electrocardiogram showed anterolateral ST and T wave abnormalities and voltage changes suggestive of left ventricular hypertrophy. An echocardiogram showed a left atrium measuring 4.1 cm, normal left ventricular size and function, left ventricular hypertrophy, and severe mitral regurgitation, for which she was treated with enalapril, isosorbide dinitrate, and propranolol. It also suggested some dilation of the inferior vena cava, raising the question of elevated right heart pressure; however, her jugular venous pressure was described as normal. Stress thallium testing was attempted, but could not be completed, as she became severely dyspneic (without chest pain). Pulmonary function studies indicated the presence of restrictive but not obstructive pulmonary disease.
She was discharged on a medical regimen, then admitted in late March 1993 for cardiac catheterization. Her cardiac hemodynamics were: RAP 7, RVP 79/9, PA 76/32, PCWP 22, LV 175/21. There was a large systolic v-wave in the PCW tracing, indicative of mitral regurgitation.1Her cardiac output was 4 L/min and cardiac index 2.3 L/min/m2(2.8 and higher are normal). Her pulmonary arterial resistance was 503, and that too is very elevated, the normal value being 100. Although left ventricular angiography was read as 3+ mitral regurgitation, I feel that it was no more than 2+. Left ventricular contractility was normal, with an ejection fraction of 71 percent, and the coronary angiogram showed essentially normal coronary arteries.
Before we get to surgery, I want to point out several things. The PCWP, representing the LAP, should be identical to the PA diastolic pressure. Her PA diastolic pressure was 32 and her PCWP was 22, with a resulting 10 mm Hg gradient across the pulmonary vascular bed. In addition, 3+ mitral regurgitation was described. Her left atrium dimension was the upper limit of normal, while her left ventricle chamber size and systolic function were completely normal. Because of the normal left ventricular and left atrial size, we know this mitral regurgitation could not have been going on for a long period of time; otherwise the left ventricle and left atrium would have been dilated. Therefore, I do not think that she had long-standing mitral regurgitation. I think that it was more acute in nature, and in my evaluation, not severe.
The patient underwent cardiac surgery for the repair of the mitral valve. The surgeon commented that the mitral leaflets did not appear to coapt centrally. There was an area of central regurgitation. The anterior tip of the anterior leaflet was rolled and abnormal in appearance, raising the question of old endocarditis.2There were no ruptured cordae tendinae, and certainly no evidence of active endocarditis, which raises the possibility of undiagnosed endocarditis being serendipitously cured by the antibiotic treatment she received in 1990 for ascending lymphangitis. The perioperative course was smooth, and a post-operative echocardiogram showed no significant mitral valve insufficiency and normal left ventricular function. One day post-operatively, the Swan-Ganz catheter was removed. With no evidence of volume overload, the final readings showed a pulmonary artery diastolic pressure of 28 mm Hg, and a PCWP of 14 mm Hg. The mitral regurgitation was repaired, but this was not her major problem.
After her operation, she was prophylactically anticoagulated with Coumadin for six months. In November of 1993, the patient presented with the complaints of dyspnea on exertion, increasing abdominal girth, and peripheral edema. She was hospitalized in early December, at which time she was described as having marked neck vein distention, abdominal distention, a right pleural effusion, epigastric and right upper quadrant tenderness, tender hepatomegaly, and 3+ peripheral edema. Rales were present. An electrocardiogram showed sinus rhythm, right axis deviation, and ST and T wave changes suggesting left ventricular enlargement. Her chest roentgenogram was consistent with left heart failure. Repeat echocardiograms both show that her left ventricular ejection fraction was 40-45 percent, abnormal but adequate. She had no significant mitral regurgitation, but mild to moderate tricuspid regurgitation. Her liver function tests were compatible with chronic passive congestion of the liver (Table 2). Anemia was present, with hemoglobins of 8-10 gram/dL, hematocrits of approximately 27-30 percent and a mean corpuscular volume of 72. Iron deficiency was confirmed by a low serum iron and ferritin level. Synthetic liver function abnormalities were present, as noted by an elevated prothrombin time, a low serum cholesterol, and a mildly depressed albumin level. Stools were guaiac negative, and a GI workup did not disclose a bleeding site. During this hospitalization the patient was treated with digitalis and diuretics. Enalapril 10 mg BID was continued, and she lost approximately 10 kg. The shortness of breath resolved very quickly, and the lungs became clear. The systemic venous congestion cleared much more slowly.
She was discharged in late December, and within two weeks came back to the Emergency Department complaining of diarrhea, vomiting, and mid-abdominal discomfort. She left the hospital and was brought back to the Emergency Department the next day by EMS, and as described in the presentation, died soon after arrival.
The patient's cardiac arrest, as described, represents electro-mechanical dissociation (also called PEA - pulseless electrical activity). By that, I mean she had electrical activity, but no mechanical activity-no blood pressure. This type of cardiac arrest is most commonly seen in acute massive pulmonary embolism, acute pericardial tamponade, hypovolemia, tension pneumothorax and myocardial rupture after myocardial infarction.3
Going back over the recent history, which spans less than two years, we conclude that her major complaint was always dyspnea, and that her chest pain was atypical. Her mitral regurgitation was described as severe, though it was not associated with significant left atrial enlargement. The left ventricular chamber size and systolic function were normal. There was a gradient across the pulmonary vascular bed at the time of catheterization prior to the surgery. I believe that the mitral regurgitation, although present, was never severe. Although I believe that the mitral valve repair was intact up to the time that she died, it seems that she had significant pulmonary hypertension soon after the operation. The follow-up echocardiograms never showed mitral regurgitation. I believe a catheterization would have demonstrated an elevated LVEDP, elevated LAP, a large gradient across the pulmonary vascular bed, severe pulmonary hypertension, right ventricular failure, and a very high right atrial pressure.
Now, what could have been the cause of her pulmonary hypertension and right heart failure? In any patient with these findings after a mitral valve repair, you have to consider acute mitral regurgitation due to valve failure. Infective endocarditis was mentioned by the students. Anything causing recurrent severe mitral regurgitation could have precipitated this picture.4However, we have no evidence for this diagnosis; trans-thoracic echocardiograms showed no mitral regurgitation, and no murmur was described. Additionally, after mitral repair, the mitral valve can be stenotic. Mitral obstruction could lead to LA hypertension, pulmonary hypertension and right heart failure. We have no evidence for this either. A third possibility is that she could have developed restrictive pericarditis related to a perioperative post-pericardiotomy syndrome or pericarditis progressing to fibrosis and restriction of myocardial function.5,6This diagnosis is rather appealing to me because it would explain the severe right-sided findings and the much less impressive left-sided findings. However, I have never seen restrictive pericarditis occur within nine months of heart surgery. Finally, we also must consider the possibility that the surgeons left an atrial septal defect, and that the patient subsequently developed a left-to-right shunt, producing signs of right heart failure.
Dr. Knowlton: What do you think ultimately caused her demise?
Dr. Heissenbuttel: Initially, I thought the most likely possibility was that this woman had multiple pulmonary emboli-that the embolic process actually began before her cardiac surgery, and recurred after the coumadin was stopped six months after the cardiac surgery. I am betting heavily that she had a final acute pulmonary embolus, and that that is what caused her demise. Having come to that conclusion, there are several points I would like you to consider.
This woman had impressive left ventricular hypertrophy, which was present in January 1993 when she was first hospitalized with cardiac symptoms. Left ventricular hypertrophy was seen on electrocardiograms and confirmed by echocardiography. I initially thought, "she was hypertensive, therefore she has cardiac hypertrophy." However, during the period from May 1989, when the electrocardiogram was normal, until 1993, the blood pressures we have were normal; she was treated and was probably not hypertensive. Therefore my original conclusion, that hypertrophy of the left ventricle was due to hypertension, was probably wrong. The hypertrophy developed over a short period of time, and it occurred while her blood pressure was well-controlled.
What else could have caused her hypertrophy? If we throw out hypertension and conclude that mitral regurgitation was not a major problem, then we have to conclude that she had a primary myocardial disease. The disease was in the muscle, not the valve, and was not related to hypertension. But whatever it was, it produced only diastolic dysfunction-a stiff heart. Her left heart, even though it was thick and non-compliant, contracted well. A cardiac catheterization during her final admission would have clarified the hemodynamics. Even then, a biopsy would have been needed to know what was occurring in the myocardium. Without tissue, a definitive diagnosis could not have been made.
The mitral regurgitation, which was due to a myxomatous valve, was moderate at best, was adequately repaired, and was not related to her death.
Dr. Knowlton: Dr. Austin, would you show us the films.
Dr. Austin: Her 1990 chest radiographs were absolutely normal, including the size of the heart. On her December 1993 chest radiograph, it is difficult to tell if there is cardiomegaly because a moderately large right-sided pleural effusion is present. You cannot see clearly on the lateral film because of motion. On her final admission in January 1994, the A-P chest radiograph taken in the Emergency Department (Figure 1) shows mild cardiomegaly, generalized mild haziness throughout the lungs (presumably from pulmonary edema), and no evidence of pleural effusion.
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Radiographically, I like to think of borderline cardiomegaly as a cardiothoracic ratio of 47-50 percent, and cardiomegaly as being around 60 percent. In my opinion, there is a mild-to-moderate group in between, which represents the breakpoint of the Starling curve. This heart, I think, is around that point; thus, we see evidence of a somewhat decompensated heart. Whether the decompensation is due to left heart or right heart failure is open to discussion.
In terms of the enlarged hilar arteries, consider the differential diagnosis of pulmonary arterial hypertension. I like to think of pulmonary hypertension as having two cardiac causes, two pulmonary parenchymal causes, and two pulmonary vascular causes. The first cardiac cause is a left-to-right shunt, which in an adult is usually an atrial septal defect (ASD). In the adult, we expect to see mild enlargement of the cardiac silhouette in an ASD. The chamber that characteristically dilates the most in this entity is the right ventricle, and we thus expect to see filling in behind the sternum, which is not present on the earlier radiograph from 1990. It does look like it was filled in 1993, but that was after a surgeon had already been in the anterior mediastinum, so one can't really tell. What we see in terms of the pulmonary vessels is that the vessels get bigger. These enlarged pulmonary vessels generally come to attention when the shunt ratio is 2.5-3.0:1.0 or more.7
Dr. Heissenbuttel: Or in someone who has been operated on, anything over 2:1 or above.
Dr. Austin: The second major cardiac cause of pulmonary hypertension is mitral stenosis, with increased back pressure affecting the pulmonary vasculature. The radiographic findings include an enlarged left atrium, and if it is late in the natural history, calcification of the mitral valve. These findings are readily seen on the plain films, better on the lateral views. To locate the mitral valve on frontal radiographs, go to the geographical center of the heart and then go 1 cm to the left. The classic imaging finding is pulmonary arterial hypertension secondary to pulmonary venous hypertension with distention of the upper vessels.
Parenchymal causes of pulmonary hypertension mean destroyed lung. The most common cause of this in our society is probably pulmonary emphysema. Pulmonary emphysema severe enough to cause pulmonary hypertension requires the destruction of a lot of lung, which we see on the plain film as severe bullous disease or extensive attenuation of vessels. Pulmonary emphysema of at least moderate severity can be picked up on plain films. The other major category of destructive lung disease is widespread pulmonary fibrosis, for example that caused by sarcoidosis, idiopathic fibrosis, and scleroderma.
Among pulmonary vessel causes of pulmonary hypertension, the most common cause is pulmonary emboli. The other major category, pulmonary vasculitis, is quite rare as a cause of pulmonary hypertension.
Dr. Knowlton: Dr. Cattoretti, would you discuss the pathologic findings.
Dr. Cattoretti: The heart was globular shaped, and weighed approximately 790 grams, twice the usual size. The mitral valve was narrowed (4.5 cm circumference) and the leaflets were sclerotic and partially fused together. The left ventricle had concentric hypertrophy and the resulting small ventricular cavity had a fibrosed, whitish endocardium. The left ventricle on section was thicker (maximum 17 mm) and firmer than expected. On trichrome stain of the heart (Figure 2), there was myocyte hypertrophy as seen in many myopathies, due to the adaptive changes of surviving muscle cells. You can see the pleomorphic nuclei and the rearrangement of myofibers. Also note the presence of amorphous material between the myocytes in the interstitium. It compresses and leads to atrophy of muscle cells in some areas.
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Besides the left ventricular hypertrophy, there were also hyaline changes in the wall of small renal arterioles, evidence of long-standing systemic hypertension. The atherosclerotic plaques on the pulmonary artery provide evidence of pulmonary hypertension. The thickened pericardium with adhesions between the two visceral and parietal leaflets, is a possible indication of previous pericarditis.
The lungs showed evidence of pulmonary emboli in the small arteries of the left lower lobe. However, these may not have pathologic significance because even patients without symptoms may be found to have pulmonary emboli on autopsy. On microscopic examination, the so-called "cardiac cells" were seen in the alveoli: these are macrophages engulfing extravasated erythrocytes and loaded with hemosiderin pigment. This is consistent with the small intraalveolar hemorrhages that occur with pulmonary hypertension. The alveolar fibrous septa were thickened; this may be related to pulmonary hypertension or may be due to the amorphous material we saw in the heart.
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The spleen was remarkable for its firmness and, on section, a glazed, waxy appearance that is typical of amyloidosis (Figure 3). On microscopic examination (Figure 4), you can see that the red and white splenic pulp is virtually replaced by a pink homogenous, amorphous material, which embeds the residual lymphoid population and fibroblasts. This material had a fibrillar texture on electron microscopy (Figure 5) and was stained by anti-lambda immunoglobulin antisera on immunofluorescence studies. However, Congo Red-stained sections did not have the apple green birefringence usually seen with amyloid. An identical picture was seen in the lymph nodes. The liver showed chronic congestion and amyloid-like material in the sinusoids.
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In the bone marrow (Figure 6), there was an increased number of plasma cells, some of them binucleated, located away from the vessels, sometimes in clusters. No atypia was seen. Immunostaining of the bone marrow was non-contributory, as is often the case on autopsy. Amyloidosis caused by deposition of light chain immunoglobulins (AL) and particularly, lambda light chain, is the most frequent form of amyloidosis and is often associated with proliferation of a B-cell subset (multiple myeloma or small lymphocytic neoplasm). The organ most frequently involved in B-cell-associated amyloidosis is the heart. Here, amyloidosis may mimic constrictive pericarditis. Macroscopically, in most cases, the heart does not show dramatic changes, but is enlarged and firm. Light chains are deposited in the tissue and tend to form beta-pleated sheets of insoluble material.
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The bone marrow findings point toward a diagnosis of amyloidosis secondary to a B-cell proliferation. The lab values are scanty, but the bone marrow picture is consistent with benign monoclonal gammopathy, a not-yet-neoplastic proliferation of bone marrow plasma cells in which a monoclonal peak is present in the serum, but major criteria for myeloma are missing. In some patients, a protein peak in the serum may be missed, particularly when the atypical plasma cells produce light chains only, which are very difficult to detect because their renal excretion occurs rapidly.
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Dr. Knowlton: Without tissue you could not have made this diagnosis.
Dr. Cattoretti: If she had, as Dr. Heissenbuttel suggested, had a second cardiac catheterization with biopsy, then this diagnosis could have been made earlier.
Dr. Heissenbuttel: The catheterization was really the essential thing to do, and it would have explained the hemodynamics. But, even if the catheterization had been repeated, we would probably not have thought to biopsy her. This lady is 48 years of age, and she died within two years of the onset of the disease.
Dr. Knowlton: Do you think that the amyloidosis killed her?
Dr. Cattoretti: It was a contributing factor, but not the most immediate cause of her death.
Dr. Heissenbuttel: Many of the cases of amyloidosis that we have treated over the years presented with elevated right heart pressure due to a restrictive cardiomyopathy. But I do not believe that this is what killed her. Although conduction defects that occur with amyloidosis can be fatal, amyloid deposition cannot explain the gradient across the pulmonary vascular bed. And the final event was an electromechanical dissociation cardiac arrest. If her cardiac arrest were due to an amyloid heart with a conduction defect or digitalis toxicity, you would expect ventricular fibrillation, and she could have been cardioverted. Therefore, something else had to have happened to her that day.
Dr. Knowlton: Do you mean pulmonary emboli?
Dr. Cattoretti: Yes, she had pulmonary emboli. In fact, she could have had a shower of pulmonary emboli. Considering the pathologic findings, the presence of EMD -of which pulmonary embolus is a potential cause-and the temporal association between these events and the discontinuation of her coumadin therapy, I would agree that her ultimate demise was related to an embolus.
Anatomic Diagnosis 1. Benign monoclonal gammopathy 2. Amyloidosis 3. Restrictive cardiomyopathy 4. Pulmonary emboli
From the College of Physicians and Surgeons and the Presbyterian Hospital in the City of New York. Departments of Medicine (R.H.H., A.I.K.), Pathology (G.C.), and Radiology (J.H.M.A.).
References