Assessment of Heart Failure: Invasive and Noninvasive Methods

Abstract

The symptoms of hypertrophic cardiomyopathy (HCM) result from a combination of high filling pressures (“backward” heart failure) and low cardiac output (“forward” heart failure). The mainstay of noninvasive testing in symptomatic patients with HCM is transthoracic echocardiography. The detection of concomitant coronary artery disease (CAD) as a cause of symptoms may be difficult by clinical assessment and exercise testing; cardiac computerized tomographic angiography (CTA) has emerged as the most useful noninvasive test for the assessment of potentially ischemic symptoms due to CAD. Cardiac catheterization is useful for documenting right-sided pressures and cardiac output, determining whether a left ventricular outflow tract (or midcavity) gradient is present at rest or with provocation, separating aortic valvular from subvalvular and supravalvular gradients, determining whether CAD is present, and excluding other pathologic causes of left ventricular thickening. Cardiopulmonary exercise testing provides quantification of functional capacity and distinguishes between cardiac and pulmonary contributions to symptoms.

Appendices

Questions

• 1. In which position should the patient be placed while Valsalva maneuver is performed?

  • A. Left lateral decubitus

  • B. Right lateral decubitus

  • C. Standing

  • D. Sitting up at 90 degrees

  • E. Supine

• 2. Which of the following correctly describes the Brockenbrough sign?

  • A. Decrease in systolic blood pressure in the beat following a VPB

  • B. Decrease in diastolic blood pressure in the beat following a VPB

  • C. Decrease in pulse pressure in the beat following a VPB

  • D. Increase in LVOT gradient in the beat following a VPB

  • E. Decrease in LVOT gradient in the beat following a VPB

• 3. A 52-year-old woman with apical HCM presents with exertional chest heaviness which has progressed over the last 6–12 months. Heart rate is 55 and blood pressure is 104/68. Electrocardiogram (ECG) shows normal sinus rhythm, left atrial enlargement, and deep T wave inversion in leads V_4–6. Holter monitor shows sinus rhythm during her symptoms. Echocardiography demonstrates apical hypertrophy up to 18 mm and no LVOT gradient at rest or with the Valsalva maneuver. Which of the following is the best next diagnostic step to further investigate the cause of her exertional chest symptoms?

  • A. Radionuclide exercise stress testing

  • B. Coronary arteriography

  • C. Exercise stress echocardiography

  • D. Dobutamine stress echocardiography

  • E. Cardiac CT angiography

• 4. A 48-year-old man with HOCM and COPD presents with worsening exertional dyspnea over the past 1–2 years. Heart rate is 65 and blood pressure is 124/78. Physical examination revealed a grade III systolic ejection murmur which is augmented by the Valsalva maneuver. Electrocardiogram (ECG) shows normal sinus rhythm, left atrial enlargement, and T wave inversion in leads I, II, III, _aV_F, and V_2–6. Echocardiography demonstrates septal hypertrophy up to 19 mm and an LVOT gradient of 34 mmHg at rest and 54 mmHg with the Valsalva maneuver. Cardiac catheterization shows right atrial pressure 14 mmHg, pulmonary arterial systolic pressure 54 mmHg and mean pressure 40 mmHg, pulmonary capillary wedge pressure 12 mmHg, cardiac output 4.4 liter/min, cardiac index 1.6 liter/min/m², and 50% stenosis of the right coronary artery. Which of the following is the most likely cause of his exertional dyspnea ?

  • A. Left-sided heart failure due to LVOT obstruction

  • B. Left-sided heart failure due to undiagnosed valvular heart disease

  • C. Intrinsic pulmonary or pulmonary vascular disease

  • D. Coronary artery disease

  • E. Deconditioning

• 5. A 48-year-old man is referred to the HCM program for suspected HCM. He is asymptomatic and actively participated in recreational sports. He has no family history of HCM. ECG shows normal sinus rhythm and left ventricular hypertrophy with nonspecific ST-T changes. Echocardiography shows mild aortic regurgitation, septal thickness 15 mm, posterior wall thickness 13 mm, ejection fraction 0.65, LVOT gradient 35 mmHg at rest without augmentation with Valsalva maneuver, trace mitral regurgitation, and estimated RV systolic pressure 28 mmHg. Which of the following is the best next diagnostic modality?

  • A. Cardiac MRI

  • B. Transesophageal echocardiography

  • C. Coronary arteriography

  • D. Cardiac CT angiogram

  • E. Exercise stress echocardiography

• 6. A 62-year-old woman is referred to our HCM program with exertional dyspnea , chest pain, and lightheadedness. She has been treated with metoprolol 100 mg PO daily, with partial improvement. Heart rate is 51 and blood pressure 105/80. There is a grade II systolic ejection murmur which is louder with the Valsalva maneuver. There is no jugular venous distension, rales, or edema. Electrocardiogram (ECG) shows normal sinus rhythm, left atrial enlargement, and T wave inversion in leads I, II, III, _aV_F, and V_2–6. Echocardiography demonstrates an LVOT gradient of 34 mmHg at rest and 84 mmHg with the Valsalva maneuver. Coronary arteriography shows no coronary atherosclerosis. She declines invasive treatment, including catheter-based and surgical interventions. Which of the following pharmacologic interventions is the best option to treat her symptoms?

  • A. Increase the dosage of metoprolol

  • B. Add verapamil

  • C. Add disopyramide

  • D. Add losartan

  • E. Add furosemide

• 7. A 58-year-old man with history of benign prostatic hyperplasia is referred to our HCM program with exertional dyspnea . Heart rate is 52 and blood pressure 120/55 while taking metoprolol 100 mg daily. He has jugular venous distention to the earlobe, bilateral rales, no murmur, and bilateral 2+ edema up to the knee. ECG shows normal sinus rhythm and deep apical T wave inversions. Echocardiography shows septal thickness 11 mm, posterior wall thickness 9 mm, apical wall thickness 18 mm, ejection fraction 0.55, no LVOT gradient at rest or during the Valsalva maneuver, biatrial enlargement, and estimated RV systolic pressure 48 mmHg. Cardiac catheterization shows right atrial pressure 14 mmHg, pulmonary arterial systolic pressure 54 mmHg and mean pressure 40 mmHg, pulmonary capillary wedge pressure 33 mmHg, cardiac output 4.4 liter/min, cardiac index 1.6 liter/min/m², and minimal coronary atherosclerosis. There is no LVOT gradient with exercise or pharmacologic provocation. Which of the following is the best treatment option to relieve his symptoms?

  • A. Furosemide

  • B. Verapamil

  • C. Disopyramide

  • D. Alcohol septal ablation

  • E. Septal myectomy

• 8. A 56-year-old man returns to the HCM program for follow-up. He has had worsening exertional dyspnea over the past few months. There is a grade III systolic murmur with wide radiation, including to the axilla; with Valsalva maneuver, it becomes harsher in quality. ECG shows sinus rhythm and left atrial enlargement. Echocardiography shows septal thickness 15 mm, posterior wall 12 mm, left ventricular end-diastolic dimension 56 mm, ejection fraction 0.75, LVOT gradient 18 mmHg at rest and 47 mmHg with the Valsalva maneuver, SAM, prolapse of the posterior mitral leaflet with moderate to severe regurgitation, left atrial enlargement, RV systolic pressure 48 mmHg, mild tricuspid regurgitation, and right atrial enlargement. There are two jets of MR, one posteriorly directed and one anteriorly directed, with the latter predominating. Coronary arteriography shows 50% stenosis of the left circumflex artery with normal fractional flow reserve. Which of the following intervention is most likely to relieve his exertional dyspnea ?

  • A. Alcohol septal ablation

  • B. Percutaneous coronary intervention

  • C. Alcohol septal ablation and percutaneous coronary intervention

  • D. Septal myectomy and possible coronary artery bypass grafting

  • E. Mitral valve repair/replacement, septal myectomy, and possible coronary artery bypass grafting

• 9. Connect diseases 1–3 in the table below with the corresponding cardiac disease in the following list:

  • A. HCM

  • B. Valvular AS

  • C. Subaortic membrane

	Site of gradient	Brockenbrough sign
Disease 1	LV → Ao	Absent
Disease 2	LV → LV	Present
Disease 3	LV → LV	Absent

• 10. Connect myocardial pathology specimens 1–4 below with the corresponding cardiac disease in the following list:

  • A. Amyloidosis

  • B. Fabry disease

  • C. HCM

  • D. Sarcoidosis

Disease 1:

Disease 2:

Disease 3:

Disease 4:

Answers

• 1. E

• In patients with HOCM , the Valsalva maneuver may result in symptomatic hypotension, sometimes causing presyncope or frank syncope. Therefore, the patients should be placed in the supine position before the maneuver is elicited.

• 2. C

• The Brockenbrough sign is a decrease in the systemic arterial pulse pressure (not an increase in LVOT gradient) in the beat following a ventricular premature beat.

• 3. E

• Exercise-induced electrocardiographic changes are nonspecific in patients with HCM. Moreover, stress radionuclide perfusion imaging may show fixed or reversible defects in patients with HCM in the absence of CAD [7]. In addition, regional differences in wall thickness may cause apparent differences in tracer uptake that do not reflect abnormalities in perfusion. The diagnostic accuracy of exercise echocardiography to detect wall motion abnormalities has not been validated in patients with HCM. Coronary CT angiography is a highly sensitive test for the presence of CAD and has emerged as the most useful noninvasive test for the assessment of potentially ischemic symptoms in due to CAD, especially in younger patients. In contrast, cardiac catheterization may be helpful in older patients, in whom CTA may be equivocal due to coronary calcification.

• 4. C

• This patient has a high transpulmonary pressure gradient (40–12 = 28 mmHg), with high pulmonary vascular resistance as well as normal pulmonary capillary wedge pressure (28/4.4 = 6.4 wood units = 509 dyn·s·cm⁻⁵), suggesting that his COPD is the cause of his dyspnea.

• 5. B

• The presence of aortic regurgitation should prompt a careful evaluation for a subaortic membrane as a mimic of HOCM . Among the listed items, transesophageal echocardopgraphy is the best diagnostic modality for detection of a subaortic membrane.

• 6. C

• Metoprolol cannot be increased and verapamil cannot be added because her heart rate is already low at 51. Losartan is relatively contraindicated in patients with LVOT obstruction and is at any rate note likely to be of benefit to this patient. Furosemide is typically reserved for patients with overt volume overload and may worsen LVOT obstruction. Disopyramide is a good medical option for symptomatic patients with HOCM who have no contraindications for disopyramide (e.g., closed-angle glaucoma, benign prostatic hyperplasia, QT prolongation, or uncontrolled hypertension).

• 7. A

• Verapamil cannot be added to this patient’s medical regimen because his heart rate is already low. Disopyramide is reserved for HOCM (this patient has a nonobstructive form of HCM). Additionally, disopyramide is relatively contraindicated in patients with benign prostate hyperplasia because its anticholinergic effects may cause urinary retention. Septal reduction therapy such as alcohol septal ablation and septal myectomy is reserved for patients with HOCM . Diuretics must be used with caution in patients with HCM, particularly if obstruction is present, but may be useful for relieving symptoms in patients with overt volume overload.

• 8. E

• This patient has intrinsic mitral valve disease concurrent with SAM provoked by LVOT obstruction. Therefore, pure septal reduction therapy (e.g., alcohol septal ablation, septal myectomy) would be insufficient to relieve his symptoms, since it would only treat the latter. Percutaneous coronary intervention would not change his symptoms given the nonobstructive nature of his coronary artery disease. Symptomatic relief of this patient requires repair/replacement of the mitral valve as well as septal reduction therapy.

• 9. Disease 1 = b, Disease 2 = a, Disease 3 = c

• Please refer to Table 7.1 in the main text.

• 10. Disease 1 = d, Disease 2 = a, Disease 3 = b

• Please refer to Fig. 7.12 in the main text.