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Open access

Sergio Barros-Gomes, Niyada Naksuk, Dragan Jevremovic and Hector R Villarraga

Cardiac amyloidosis (CA) is relatively rare and frequently misdiagnosed. Other disorders presenting with increased left ventricular (LV) mass can mimic its diagnosis. This case illustrates unique findings of primary light chain (AL) amyloidosis in a patient with remarkable signs of CA. Here, we report a 49-year-old male with prior diagnosis of hypertrophic cardiomyopathy (HCM) based on an echocardiogram performed 1 year earlier, which presented with 8 weeks of periorbital rash. The patient had numbness in the past 3 years. More recently, the patient presented with shortness of breath. Physical examination was remarkable for periorbital purpura, macroglossia and orthostatic hypotension. Cardiac auscultation showed S3 and S4. Electrocardiography showed diffuse low-voltage QRS complexes. Echocardiography revealed severe diastolic impairment; granular ‘sparkling’ pattern of the myocardium with thickened walls, interatrial septum and valves; and pericardial effusion. Diastolic dysfunction and thick walls with low ECG voltage are compelling diagnostic findings. Laboratory workup showed increased free light chain-differential (FLC-diff), N-terminal fragment of brain natriuretic peptide (NT-BNP) and cardiac Troponin T (cTnT). Bone marrow biopsy confirmed AL amyloidosis. A diagnosis of AL amyloidosis with cardiac involvement mimicking HCM was made. The patient died during hospitalization due to sudden cardiac death. This case illustrates the importance of the combination of clinical, serological, and electro- and echocardiographic findings to establish the diagnosis of CA.

Learning points:

  • Several disorders presenting with increased LV mass can mimic CA.

  • Echocardiography is one of the most important methods to diagnose CA and HCM.

  • Signs of CA include LV wall thickness; thickening of interatrial septum, valves and right ventricular free wall; and pericardial effusion. Diastolic dysfunction and thick walls on echocardiography with low ECG voltage are the hallmark of disease.

  • CA is a major prognostic factor in AL amyloidosis.

  • Signs of HCM on echocardiography include several patterns of LV hypertrophy, such as sigmoidal, reverse curve, neutral and apical morphologies; LV outflow tract or mid-cavity obstruction; systolic anterior motion of mitral leaflets; mitral regurgitation and diastolic dysfunction.

  • The combination of clinical and serological features, along morphological and functional structures, has an important role for establishing diagnosis and predicting prognosis.

Open access

Manasawee Indrabhinduwat, Maria C Arciniegas Calle, Joseph P Colgan and Hector R Villarraga

Summary

Thirty-seven-year-old male presented with cough, dyspnea, significant weight loss (20 kg) and subacute fever for the past 2 months. Physical examination revealed inspiratory and expiratory wheezing bilaterally. A normal S1, S2 and a 3/6 systolic ejection murmur at the left upper parasternal border with respiratory variation were found during cardiac auscultation. Kidney and bone marrow biopsy reported a high-grade B-cell lymphoma. Echocardiography and cardiac CT findings consisted of multiple intracardiac masses affecting the right ventricular (RV) outflow track, RV apex, medial portion of the right atrium and posterior left atrium, as well as mild impairment of the RV systolic function. The masses in the RV outflow track caused partial obstruction (pulmonary valve peak velocity 2.3 m/s) with a RV systolic pressure of 43 mmHg. The infiltrative mass in the interatrial septum extended into both the right and left atrial cavities. The right superior pulmonary vein was occluded. This patient was treated with aggressive chemotherapy and had a good clinical response that resulted in mass size reduction after the first course of chemotherapy. Multimodality imaging techniques such as echocardiography, cardiac CT and PET scan can provide complementary information to better evaluate, stage and manage these patients.

Learning points:

  • Lymphoma can be found as a primary tumor in cardiac tissue, but secondary cardiac lymphoma is far more common.

  • Appropriate investigation, histopathology, immunophenotype, staging and risk assessment are required for definite diagnosis and treatment.

  • Cardiac lymphoma frequently manifests as an ill-defined, infiltrative mass. Typical location is in the atrium (right atrium is the most common site). Pericardial thickening or effusion is also common.

  • Echocardiography is a quick, bedside, non-invasive assessment of anatomical involvement and hemodynamics affected by cardiac lymphoma. Echocardiographic findings of cardiac lymphoma include a hypoechoic, ill-defined infiltrative masses in the myocardium, nodular protrusion into cardiac chambers and pericardial effusion. Obstruction of inflow/outflow track can also be found.

  • If a diagnosis of cardiac lymphoma is made, the most effective treatment is chemotherapy. Surgical treatment may have a role when hemodynamic compromise does not respond to chemotherapy and radiotherapy.

Open access

Garvan C Kane, Arun Sachdev, Hector R Villarraga, Naser M Ammash, Jae K Oh, Michael D McGoon, Patricia A Pellikka and Robert B McCully

Aim

It is not well known if advancing age influences normal rest or exercise pulmonary artery pressures. The purpose of the study was to evaluate the association of increasing age with measurements of pulmonary artery systolic pressure at rest and with exercise.

Subjects and methods

A total of 467 adults without cardiopulmonary disease and normal exercise capacity (age range: 18–85 years) underwent symptom-limited treadmill exercise testing with Doppler measurement of rest and exercise pulmonary artery systolic pressure.

Results

There was a progressive increase in rest and exercise pulmonary artery pressures with increasing age. Pulmonary artery systolic pressures at rest and with exercise were 25±5mmHg and 33±9mmHg, respectively, in those <40 years, and 30±5mmHg and 41±12mmHg, respectively, in those ≥70 years. While elevated left-sided cardiac filling pressures were excluded by protocol design, markers of arterial stiffness associated with the age-dependent effects on pulmonary pressures.

Conclusion

These data demonstrate that in echocardiographically normal adults, pulmonary artery systolic pressure increases with advancing age. This increase is seen at rest and with exercise. These increases in pulmonary pressure occur in association with decreasing transpulmonary flow and increases in systemic pulse pressure, suggesting that age-associated blood vessel stiffening may contribute to these differences in pulmonary artery systolic pressure.