Cardiovascular and Pulmonary Pathology in Systemic Disease
Henry D. Tazelaar and Marie-Christine Aubry
Many diseases affect both the heart and lungs, some in unique ways, and others in ways that are not as
specific and require clinical correlation for accurate diagnosis.
This short course utilizes eight cases which will form the basis of the discussions. Systemic and
inherited diseases in which both heart and lung are involved, and diseases in which either organ are
involved will be covered. Diagnostic criteria for specific diseases as well as criteria for specific
manifestations in each organ will be discussed. Pathologic features useful for differential diagnosis
will be highlighted when appropriate. Recognition of the specific features of these diseases may be
critical to proper patient management. A copy of the text syllabus and a PDF of the Powerpoint
presentations are available by clicking on the cases listed below.
Click on each Case number below to display the text and references for that case
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A 29-year-old woman was seen at 34 weeks of gestation for polyhydramnios. The main abnormality
identified at fetal echography was a large homogeneous echogenic mass involving the entire left
ventricular wall and interventricular septum. At 35 weeks gestation, the patient presented in active
labor and gave birth to an infant, pronounced dead thirty minutes after delivery. An autopsy of the
infant was performed. The left ventricle of the heart contained multiple masses ranging in size
between 0.6 and 5.3cm in diameter.
A 38-year-old woman presented with spontaneous pneumothorax. She had a history of subependymal
calcification, dermal angiofibromas and bilateral renal tumors. A pulmonary computed tomography scan
showed cystic lesions, involving predominantly the lower lobes. Occasional small nodules were also
identified. A wedge biopsy of the lung was performed.
A 34-year-old woman, with a 10-year history of systemic lupus erythematosus, presented with hemoptysis
and bilateral pulmonary infiltrates. She underwent a pulmonary wedge biopsy. During the course of
her work-up and treatment, she died suddenly in the hospital. An autopsy was performed.
A 35-year-old man was referred from the local prison for further evaluation of his known cardiac and
pulmonary problems. He had a history of Hodgkin disease 18 years previously. His recent history
began one month prior to admission at which time he collapsed playing basketball. At this time, he
was noted to have significant bradycardia and required a permanent pacemaker. An echocardiogram
revealed an ejection fraction of 30% and normal coronary arteries. He was discharged, but he returned
a few days later with lethargy, chest pain, and shortness of breath. He underwent bronchoscopy, at
which time, non-necrotizing granulomas were identified on biopsy, and a diagnosis of sarcoidosis
established. He was placed on 60 mg of prednisone and was then referred to Mayo for consultation. At
the time of admission, he said he was asymptomatic and felt strong. He was scheduled for a repeat
chest x-ray, EKG, and echocardiogram, as well as pulmonary function testing. He returned to the
prison and three days later was found unresponsive in the weight room. The paramedics were unable to
resuscitate him and he was pronounced dead.
A 58-year-old man was noted to have a tender swollen proximal interphalangeal joint of his right long
finger on a routine physical examination. This was treated with corticosteroids. He returned two
months later complaining of pain and swelling in multiple joints. A chest radiograph at the time
showed bi-basilar infiltrates suggestive of pulmonary fibrosis. He underwent open lung biopsy for
A 58-year-old man with a history of sarcoidosis presented with persistent cough and symptoms of
bronchitis. These had been recurrent over the past ten years and developed approximately six months
following a diagnosis of Crohn's disease of the colon. Throughout the past ten years, he has had a
persistent cough productive of five to eight teaspoons of thick, tan, yellow, "chunky" sputum and has
had numerous hospitalizations for pulmonary infections with fever and chills. He underwent an open
lung biopsy because his sputum was growing aspergillus and the possibility of invasive aspergillosis
A 19-year-old woman presented three months following a stroke at which time she was found to have an
antiphospholipid antibody (APA). At the time of evaluation for the APA, she was noted to have
numerous freckles on her face and lips and a periumbilical soft-tissue mass. Further work-up included
an echocardiogram, at which time multiple mobile intracardiac masses were identified involving the
left atrium and ventricle, and right ventricle. She underwent surgical excision of both the cardiac
and soft-tissue mass. Your section is from the right ventricular mass.
A 58-year-old woman was admitted to the hospital for shortness of breath and chest pain. Her
electrocardiogram showed atrial fibrillation without rapid response. The ST segment was normal and
there was no Q wave. Her cardiac enzymes were normal. An angiogram was performed and showed no
significant coronary atherosclerosis. An echocardiogram revealed a restrictive cardiomyopathy. An
endomyocardial biopsy of the right ventricle was performed.
A 73 year-old gentleman presented at the emergency room with vague chest pain. He has a past history
of Wegener's granulomatosis and mild well controlled systemic hypertension. He was evaluated for
acute ischemic heart disease. He underwent a stress electrocardiogram (ECK) and echocardiogram. The
stress ECG was negative and the stress echocardiogram revealed a severe concentric hypertrophy of the
left ventricle with severe hypokinesis of the inferior wall of the left ventricle, which remained
unchanged during effort. His troponin was negative. The chest pain was considered of non-coronary
origin and he was referred to cardiology for further work-up of the ventricular hypertrophy. A
cardiac catheterization confirmed the absence of significant coronary disease and a right
endomyocardial biopsy was performed.