Manfred Ritter

Isolated Noncompaction of the Myocardium in Adults

OBJECTIVE To describe the entity of isolated ventricular noncompaction (IVNC) and present a series of cases of this rare disorder in an adult population. MATERIAL AND METHODS We review a 10-year experience with the diagnosis of IVNC and discuss the clinical, echocardiographic, and pathologic features of this condition. Echocardiographic diagnostic criteria included the absence of coexisting cardiac abnormalities, the presence of prominent and excessive trabeculations of one or more ventricular wall segments, and intertrabecular spaces perfused from the ventricular cavity. Pathologic examination focused on regions with exaggerated trabeculations and deep intertrabecular spaces. RESULTS IVNC is an unexplained arrest of myocardial morphogenesis previously encountered mainly in pediatric patients. Among 37,555 transthoracic echocardiographic studies performed at our hospital between January 1984 and October 1993, 17 cases of IVNC were identified in adult subjects (14 men and 3 women, 18 to 71 years of age). The mean time from onset of symptoms to correct diagnosis was 3.5 +/- 5.7 years, and the mean duration of follow-up was 30 +/- 28 months. Common clinical symptoms were heart failure, ventricular arrhythmias, and a history of embolic events. Two-dimensional echocardiography revealed 10 patients with left ventricular and 7 (41%) with biventricular IVNC. During a 6-year follow-up period, eight patients died and two underwent heart transplantation. CONCLUSION Although the diagnosis of IVNC in an adult population is often delayed because of similarities with more frequently diagnosed conditions, two-dimensional echocardiography will facilitate the diagnosis of IVNC in this subset of patients. Because of the high incidence of heart failure, ventricular arrhythmias, and embolization in adults with IVNC, early diagnosis is important.

Prevention of High-Altitude Pulmonary Edema by Nifedipine

Abstract Background. Exaggerated pulmonary-artery pressure due to hypoxic vasoconstriction is considered an important pathogenetic factor in high-altitude pulmonary edema. We previously found that nifedipine lowered pulmonary-artery pressure and improved exercise performance, gas exchange, and the radiographic manifestations of disease in patients with high-altitude pulmonary edema. We therefore hypothesized that the prophylactic administration of nifedipine would prevent its recurrence. Methods. Twenty-one mountaineers (1 woman and 20 men) with a history of radiographically documented high-altitude pulmonary edema were randomly assigned to receive either 20 mg of a slow-release preparation of nifedipine (n = 10) or placebo (n = 11) every 8 hours while ascending rapidly (within 22 hours) from a low altitude to 4559 m and during the following three days at this altitude. Both the subjects and the investigators were blinded to the assigned treatment. The diagnosis of pulmonary edema was based on chest radio...

Vasoconstriction of stenotic coronary arteries during dynamic exercise in patients with classic angina pectoris: reversibility by nitroglycerin.

To study the vasomotility of normal and diseased coronary arteries during dynamic exercise, symptom-limited supine bicycle exercise during cardiac catheterization was performed by 18 patients with classic angina pectoris. The cardiovascular response was assessed by hemodynamic measurements and computer-assisted determination of normal and stenotic coronary artery luminal areas from biplane coronary angiograms made before, during, and after exercise. After baseline measurements were recorded, 12 patients (group 1) performed bicycle exercise for 3.4 min (mean), reaching a maximum workload of 81 W (mean); at the end of exercise they received 1.6 mg sublingual nitroglycerin. After measurements at rest in six other patients (group 2), 0.1 mg intracoronary nitroglycerin was given, followed by exercise (3.8 min, 96 W; NS) and sublingual nitroglycerin as in group 1. During exercise in group 1, luminal area of the coronary stenosis decreased to 71% of resting levels (p less than .001), while area of the normal coronary artery increased to 123% of control (p less than .001). After sublingual nitroglycerin at the end of exercise, area of the normal vessel further increased to 140% of control (p less than .001), while luminal area of the stenosis dilated to 112% of resting levels (p less than .001 vs exercise, NS vs rest). Pretreatment with intracoronary nitroglycerin increased both normal (121%; p less than .05) and stenotic (122%; p less than .05) luminal areas, while preventing the previously observed narrowing of stenosis during exercise (114%; NS). Exercise resulted in a similar heart rate-systolic pressure product and caused angina pectoris in two-thirds of the patients in each group. However, patients pretreated with intracoronary nitroglycerin (group 2) had a lower mean pulmonary arterial pressure during maximum exercise (35 mm Hg) than those patients (group 1) not receiving pretreatment (47 mm Hg; p less than .001). Group 2 patients reached a percentage of their predicted work capacity (65%) that was about the same as that during previous upright bicycle exercise (71%; NS), while group 1 patients had a significantly lower work capacity (51% of predicted) than that before catheterization (82%; p less than .001). Hence, narrowing of coronary artery stenosis during dynamic exercise is attributable to active vasoconstriction due to its reversibility by preexercise intracoronary nitroglycerin. Patients who did not experience narrowing of stenosis during exercise (group 2) had less evidence of myocardial ischemia (lower mean pulmonary arterial pressure) and maintained their work capacity.(ABSTRACT TRUNCATED AT 400 WORDS)

Nifedipine for high altitude pulmonary oedema

In a laboratory at 4559 m six subjects with high altitude pulmonary oedema (HAPO) characterised by clinical signs, severe hypoxaemia, widened alveolar-arterial oxygen gradient, pulmonary hypertension, and alveolar oedema on chest radiography were treated with nifedipine. Despite continued exercise at the same altitude this treatment, without supplementary oxygen, resulted in clinical improvement, better oxygenation, reduction of alveolar arterial oxygen gradient and pulmonary artery pressure, and progressive clearing of alveolar oedema. Nifedipine offers a potential emergency treatment for HAPO when descent or evacuation is impossible and oxygen is not available. The findings also suggest that hypoxic pulmonary hypertension is essential in the pathogenesis of HAPO.

Diastolic properties of the normal left ventricle during supine exercise.

Diastolic function in response to dynamic exercise was studied by biplane left ventriculography and by measuring left ventricular pressure with a high fidelity micromanometer tipped catheter at rest and during supine bicycle exercise in nine normal subjects. During exercise there was a fall in end systolic volume, in the time constant of left ventricular isovolumic pressure decay, and in the lowest diastolic pressure. Stroke volume, peak filling rate, mean passive filling rate, and the volume at the lowest diastolic pressure increased. There was an increase in the number of time constants that had elapsed before the lowest diastolic pressure was reached and the slope of the pressure-volume curves during passive filling (delta P/delta V) increased without changes in end diastolic pressure and volume. These results show that during exercise elastic recoil is enhanced and left ventricular relaxation is faster and more complete. Both phenomena reduce the lowest diastolic filling pressure. The observed increase in chamber stiffness from rest to exercise is probably related to increased resistance of the left ventricular wall caused by higher passive filling rates. The enhanced early diastolic pressure decay during exercise allows stroke volume to increase despite an increase in diastolic viscoelastic resistance and chamber stiffness.

Coronary reserve in patients with aortic valve disease before and after successful aortic valve replacement

In patients with aortic valve disease and normal coronary angiograms coronary reserve was determined by the coronary sinus thermodilution technique. Three groups of patients were studied: 37 preoperative patients; 18 different patients 12-52 months after aortic valve replacement and seven control subjects with no cardiac disease. Coronary flow ratio (dipyridamole/rest) was diminished in preoperative compared with postoperative patients (1.66 +/- 0.44 vs 2.22 +/- 0.85; P less than 0.05) as well as with controls (2.80 +/- 0.84; P less than 0.01), and corresponding coronary resistance ratio (dipyridamole/rest) was higher in preoperative patients than in both other groups (0.61 +/- 0.17 vs 0.48 +/- 0.14; P less than 0.05 vs 0.37 +/- 0.10; P less than 0.01). Differences in the flow ratio, but not in the resistance ratio, were significant (P less than 0.05) in patients after aortic valve replacement compared with controls. Total coronary sinus blood flow at rest was elevated in preoperative compared with both postoperative patients and controls (252 +/- 99 vs 169 +/- 63; P less than 0.01; vs 170 +/- 35 ml.min-1, P less than 0.05), whereas flows after maximal vasodilation did not differ among the three groups (416 +/- 184 vs 361 +/- 150 vs 488 +/- 235 ml.min-1). Postoperative patients showed a distinct, though not total regression of left ventricular angiographic muscle mass index and wall thickness. Nine of the 18 postoperative patients showed a normal coronary flow reserve and nine showed subnormal response. These two subgroups did not differ with respect to preoperative macroscopic and microscopic measures of hypertrophy. Thus in aortic valve disease, the reduced coronary vasodilator capacity is mainly due to an elevated coronary flow at rest, while the maximal coronary blood flow achieved is identical to that of postoperative patients and controls. With regression of left ventricular hypertrophy, flow at rest decreases and this leads to a distinct improvement of coronary flow reserve.

Quantification of mitral regurgitation with amplitude-weighted mean velocity from continuous wave Doppler spectra.

Aortic regurgitant fraction (RFao) was quantified by estimating the ratio of the forward blood flow through the aortic (Qao) and pulmonary (Qp) valve: RFao = 100(Qao - Qp)/Qao. Aortic and pulmonary flow were measured by the systolic time integrals of the amplitude-weighted mean velocity from continuous wave Doppler spectra recorded over the aortic and pulmonary valves. Thus, measurements are independent of the left and right ventricular outflow tract area. In 20 normal subjects, aortic regurgitant fraction ranged between -2.9% and +12.0% (mean +4.3%), the physiologic value being +2%. In 20 patients with pure aortic regurgitation, aortic regurgitant fraction obtained by Doppler spectra (y) was compared with that calculated from biplane left ventriculography and cardiac output determined with the Fick method (x). The correlation was r = 0.94, (SEE = 5.4%, which is 10.6% of the angiography-Fick mean value). The regression line was y = 0.87x + 6.6 (mean y = 51.2%, mean x = 51.1%). It is concluded that determination of aortic regurgitant fraction in pure aortic regurgitation by using the amplitude-weighted mean velocity from continuous wave Doppler spectra is accurate and allows easy noninvasive evaluation of the regurgitant fraction in routine clinical applications.

Pulsus alternans: Its influence on systolic and diastolic function in aortic valve disease

Left ventricular high fidelity pressure measurements and simultaneous biplane cineangiocardiography were performed in 12 patients with severe aortic valve disease (aortic stenosis in 10, aortic insufficiency in 1 and combined valve lesion in 1). Left ventricular contractility was estimated from maximal rate of left ventricular pressure rise (max dP/dt), peak measured velocity of contractile element shortening (Vpm) and mean circumferential fiber shortening velocity. Left ventricular relaxation was assessed in 12 patients from the time constant (T) of the decline in left ventricular pressure; this constant was calculated from a nonlinear regression analysis of pressure and time (method 1) and a linear regression analysis of pressure and negative dP/dt (method 2). Left ventricular diastolic function was evaluated in nine patients from simultaneous diastolic pressure-volume relations during the strong and weak beats. During pulsus alternans, heart rate and left ventricular end-diastolic pressure remained unchanged, whereas peak systolic pressure (220 versus 204 mm Hg, p less than 0.01) and end-systolic pressure (101 versus 95 mm Hg, p less than 0.01) were significantly higher during the strong beat than during the weak beat. Max dP/dt was alternating (2,162 versus 1,964 mm Hg, p less than 0.05), whereas the peak velocity of contractile element shortening remained unchanged (1.21 versus 1.18 ML/s). Systolic shortening of the left ventricular minor axis was significantly (p less than 0.02) greater during the strong (24%) than during the weak (19%) beat, but that of the left ventricular major axis remained essentially unchanged (8 versus 7%).(ABSTRACT TRUNCATED AT 250 WORDS)

Pseudoaneurysm following aortic homograft: clinical implications?

OBJECTIVE--To determine the prevalence of pseudoaneurysm formation after aortic (left ventricular outflow tract) homograft implantation and to evaluate predisposing factors. METHODS--Echocardiographic data were analysed in 30 patients for evidence of pseudoaneurysm formation after homograft implantation. Pseudoaneurysm was characterised as a perfused echo-free space between the homograft and the native aortic wall communicating with the left ventricular outflow tract. Clinical data were analysed for potential predisposing factors for pseudoaneurysm formation. RESULTS--Pseudoaneurysms were found in 22 of 30 patients. Mean age, length of follow up after surgery, aortic systolic pressure gradient (15 (SD 12) v 10 (4) mm Hg), aortic root diameter, and size of the homografts were comparable in patients with and without pseudoaneurysm. preoperative infection, operating techniques, and whether first or reoperation did not affect pseudoaneurysm formation. However, pseudoaneurysms were often localised at the site of an abscess or a paravalvular leak after eradicated prosthetic valve endocarditis. CONCLUSIONS--(1) Doppler echocardiography demonstrates that pseudoaneurysm formation is common after aortic homograft implantation. (2) A prospective study is needed to clarify the prognostic importance of pseudoaneurysms. (3) The high incidence of pseudoaneurysm formation may lead to an improvement of surgical technique (application of fibrin glue).

Retrograde ascending aortic dissection: a diagnostic and therapeutic challenge.

Aortic dissection with an entrance tear in the transverse aorta is generally considered to have the highest acute fatality rate of any type of dissection and the direction of its extension is the most difficult to predict. In a prospective study, we evaluated 61 consecutive patients (mean age 56.7 years, ranging from 21 to 75 years), presenting with ascending aortic dissection during a 36-month-period and tried to clarify the incidence of retrograde ascending aortic dissection. In 49 patients (80.3%), the intimal tear was located in the ascending aorta, whereas the dissection originated in the transverse aorta in 12 patients (19.7%); in this latter group, extension was strictly retrograde in 5 patients and in both directions in 7 patients. Three patients died before operation; 58 patients underwent aortic replacement/repair under moderate hypothermia; if the primary tear extended into the transverse aorta or was not found in the ascending aorta, the aortic arch was explored during a brief period of deep hypothermic circulatory arrest. The overall operative mortality was 12.1% (7/58); it was 10.4% (5/48) in ascending aortic dissection and 20% (2/10) in dissection of the transverse aorta. Age (P < 0.005), concomitant coronary artery disease (P < 0.01) and the site of intimal tear (P < 0.01) were significant predictive factors of operative risk. A tear in the transverse aorta is almost always associated with retrograde dissection and may simulate dissection with the entrance tear in the ascending aorta. Localization of the entrance tear remains a diagnostic challenge in aortic dissection but Doppler-echocardiography had a high sensitivity in this series (96.7%).(ABSTRACT TRUNCATED AT 250 WORDS)