Congestive heart failure in hypertensive patients with normal systolic function and dynamic left ventricular outflow obstruction.

BACKGROUND: Hypertensive patients with left ventricular hypertrophy and normal systolic function can develop congestive heart failure refractory to conventional drug therapy with digoxin, diuretic, and vasodilators. METHODS: We studied 8 patients with a history of systemic hypertension (6 females and 2 males, mean age 69+/-6 years), affected by New York Heart Association (NYHA) class IV congestive heart failure notwithstanding conventional drug therapy with digoxin, diuretic, and vasodilators. After clinical history and physical examination, blood chemistry including cardiac enzymes, arterial blood gases, chest roentgenogram, standard 12-lead ECG, and complete echocardiographic study were performed in all patients. RESULTS: In all cases, a left ventricle with increased wall thickness, normal cavity size, and normal or supernormal systolic function was shown. All patients had left ventricular systolic dynamic obstruction, with peak gradient between 36 and 130 mmHg (mean 83+/-31). After having stopped treatment with nitrates, digoxin, and diuretics, drug therapy with calcium channel antagonists or beta-blockers was started, and rapid clinical improvement with disappearance of left ventricular outflow obstruction was observed. CONCLUSIONS: Sometimes, a distinction between several forms of heart failure is clinically impossible. However, when conventional therapy is not effective in patients with longstanding history of systemic hypertension and ECG signs of left ventricular hypertrophy, diastolic heart failure and/or dynamic left ventricular obstruction should be suspected. Thus, an early echocardiographic study should be performed.

Systemic sclerosis (scleroderma). A case of recovery of cardiomyopathy after vitamin E treatment.

A 60-year-old woman with systemic sclerosis, systemic hypertension, and chronic renal failure, presented with clinical manifestations of heart failure. An echocardiogram showed a mildly dilated left ventricle and global hypokinesis. A six-month treatment including reduced sodium intake, furosemide, and nifedipine did not change the clinical and instrumental findings. Casually, vitamin E (600 mg daily) was added. After 6 months, clinical manifestations of heart failure were disappeared and the echocardiogram showed a normally-sized left ventricle with normal wall motion.

Cardiac involvement in patients with Wegener's granulomatosis.

Wegener's granulomatosis (WG) is a systemic necrotizing vasculitis, which could potentially affect any organ system. However, there have only been a few reports on cardiac involvement. We described the echocardiographic findings in nine patients affected by WG. A complete M-mode, two-dimensional, Doppler and color-Doppler transthoracic echocardiogram was performed in nine patients (seven females and two males) affected by WG. In each patient, cardiac abnormality, for example, valvular damage, left ventricular global systolic dysfunction, or pericardial effusion, was detected. In particular, heart valve disease was found in eight patients, and in three cases, aortic valve insufficiency, which was severe enough to require surgical valve replacement, was observed. Cardiac involvement in patients with WG is common. In particular, there is a high frequency of aortic valve abnormalities. Thus, an echocardiographic study should be routinely performed.

Noninvasive assessment of myocardial involvement in patients with systemic sclerosis: role of signal averaged electrocardiography.

OBJECTIVE: To assess the role of late ventricular potentials (LVP) in detecting early myocardial involvement in patients with systemic sclerosis (SSc). METHODS: Seventy-seven patients with SSc (68 women, 9 men, mean age 50 +/- 13 yrs) and 33 control subjects (18 women, 15 men, mean age 46 +/- 10 yrs) underwent resting electrocardiogram (ECG), 24 h Holter monitoring, complete echocardiographic and Doppler echocardiographic examination, and signal averaged ECG at high pass setting of 40 Hz, with the low pass fixed at 250 Hz. Patients with SSc underwent resting myocardial scintigraphy and radionuclide angioventriculography. RESULTS: The prevalence of LVP at 40 Hz was 20.5%. Compared to control subjects, patients with SSc showed higher prevalence of septal infarction pattern (p = 0.05), complex ventricular arrhythmias (p = 0.03), pulmonary arterial hypertension (p < 0.001), and LVP (p = 0.02). Forty-four patients with SSc (57.1%) had resting perfusion defects by myocardial scintigraphy. Fourteen of 15 patients with LVP showed perfusion defects compared to 29 of 58 without LVP (p = 0.002). Linear regression analysis showed that myocardial perfusion defect score was significantly correlated to either the filtered QRS duration, or the duration of low amplitude signals < 40 microV of the terminal QRS, or the root mean square voltage of the last 40 ms of the QRS complex. After a mean followup period of 20 months, 8 patients died. In 2 patients who died suddenly, LVP were present. CONCLUSION: Signal averaged ECG is a sensitive and inexpensive technique in the clinical assessment and followup of patients with SSc.