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[Pulmonary hypertension and right ventricular failure. Part III. Patients with congenital heart disease].

In a series of articles the authors discuss literature data concerning epidemiology of pulmonary hypertension, its modern classification, peculiarities of its pathogenesis and treatment in various diseases and conditions. In the third lecture they consider peculiarities of pathogenesis of pulmonary hypertension and right ventricular failure in patients with congenital heart defects, and present literature data on drug treatment and other curative interventions in pulmonary hypertension in patients with Eisenmenger syndrome.

Heart Defects, Congenital↗

Extracorporeal right to left atrial bypass to treat right ventricular failure.

Graft right ventricular failure after heart transplantation, secondary to preoperative functional pulmonary hypertension, was successfully managed in a 49-year-old patient using an extracorporeal right to left atrial bypass. We comment on the case and discuss the type of mechanical assistance used.

Cardiomyopathy, Dilated↗

Right ventricular infarction: the role of echocardiography.

Echocardiographic evaluation of patients with right ventricular (RV) ischemia and infarction has a clear clinical relevance, because the presence of RV infarction is associated with a highly increased early in-hospital mortality, which can be dramatically reduced by successful early reperfusion. However, infarcted RV did recover its function regardless of the infarcted artery patency. This observation suggests that RV myocardium almost always remains viable. This article illustrates the clinical value of echocardiography, including the transthoracic and transesophageal approaches, Doppler, and three-dimensional ultrasound, for the assessment of RV dimensions, shape, and global and regional function, and the estimation of right atrial involvement that in cases of ischemia may further modulate RV performance. In addition, the use of stress echocardiography to detect RV ischemia and evaluate the viability of infarcted RV myocardium is discussed.

Animals↗

Myocardial scars determined by delayed-enhancement magnetic resonance imaging and positron emission tomography are not common in right ventricles with systemic function in long-term follow up.

OBJECTIVE: To test the hypothesis that myocardial scars are common in patients with systemic right ventricles. METHODS: 27 consecutive patients with systemic right ventricle were studied with delayed-enhancement magnetic resonance imaging and positron emission tomography. Of the 27 patients, 18 had had an atrial switch operation a mean of 21.8 (SD 4.5) years previously and were 23.4 (SD 5.3) years old. Nine patients without previous heart surgery had congenitally corrected transposition of the great arteries and were 35.3 (SD 15.6) years old. RESULTS: Only one patient had a subendocardial scar identified by delayed-enhancement magnetic resonance imaging. Positron emission tomography identified no myocardial scars. CONCLUSIONS: This study shows that the hypothesis that myocardial scars are common in patients with systemic right ventricles is not correct.

Adolescent↗

[Effectiveness of adenosine as a pulmonary vasodilator during weaning from extracorporeal circulation after mitral valve replacement in a patient with severe pulmonary hypertension].

A 75-year-old woman with mitral stenosis and tricuspid insufficiency underwent mitral valve replacement surgery. After insertion of a catheter into the pulmonary artery, upper respiratory system pressure was seen to rise above systemic arterial pressure. To relax the pulmonary vessel, we administered adenosine, a drug that is metabolized rapidly during its first pass through the lungs before hypotension or other adverse side effects occur systemically. Adenosine decreased pulmonary artery pressure and vascular resistance, optimizing right cardiac function with successful output to extracorporeal circulation.

Adenosine↗

[Inferior infarct with associated anterior ST elevation: an unusual presentation of right ventricular involvement. Description of three cases and a brief review of the problem].

ST segment elevation in the left precordial leads in the setting of an acute inferior myocardial infarction may represent an unusual electrocardiographic pattern of right ventricular infarction. We present our experience about three patients with first inferior acute myocardial infarction in whom concomitant anterior ST segment elevation was observed. All patients were submitted to urgent coronary angiography because of repeated episodes of myocardial ischemia (case 1) or hemodynamic derangement (case 2, 3) with hypokinetic arrhythmias (case 3). In all patients 2D echocardiographic examination performed before angiography showed a dilated, hypo-akinetic right ventricle and wall motion abnormalities only in inferior, posterior and/or lateral segments of the left ventricle. Proximal right coronary occlusion was found in all patients, and coronary angioplasty was successfully attempted in all but one case. In patients with first inferior myocardial infarction, left precordial ST segment elevation mimicking an anterior infarction may be the less frequent ECG pattern of right ventricle ischemic involvement. Routine right chest leads and early echocardiographic examination allow to identify the patients with right ventricle infarction and concomitant anterior ST segment elevation. In these patients, early and correct diagnosis is important in order to choice the appropriate therapeutic pathway.

Coronary Angiography↗

Is thrombolytic therapy effective for pulmonary embolism?

Pulmonary embolism is a disorder that is associated with significant morbidity and mortality. Right-sided heart failure and recurrent pulmonary embolism are the main causes of death associated with pulmonary embolism in the first two weeks after the embolic event. Thrombolysis is a potentially lifesaving therapy when used in conjunction with standard anticoagulation. However, it has significant side effects and must therefore be used with caution. Indications for thrombolysis are not well defined and are thus controversial. The only current absolute indication is massive pulmonary embolism with hypotension. Other potential indications include right heart dysfunction, recurrent pulmonary embolism and the prevention of pulmonary hypertension. However, no evidence exists to show benefit of thrombolytic therapy over standard anticoagulation therapy for recurrent pulmonary embolism, mortality or chronic complications. Bleeding is the most common complication of thrombolysis and may be fatal.

Anticoagulants↗

Right ventricular function: a comparison between blood and crystalloid cardioplegia.

Blood cardioplegia resulted in better left ventricular (LV) function than crystalloid cardioplegia after elective coronary artery bypass operations. However, most methods of cardioplegic delivery may not adequately cool and protect the right ventricle, and right ventricular (RV) dysfunction may limit hemodynamic recovery. Therefore, RV and LV temperatures were measured intraoperatively and RV and LV function were evaluated postoperatively in 80 patients with double-vessel or triple-vessel coronary artery disease who were randomized to receive either blood cardioplegia or crystalloid cardioplegia. Myocardial performance, systolic function, and diastolic function were assessed with nuclear ventriculography by evaluating the response to volume loading. Preoperatively the groups were similar. Intraoperatively, blood cardioplegia resulted in significantly warmer LV and RV temperatures (left ventricle: 15.5 degrees +/- 0.2 degrees C with blood cardioplegia and 12.6 degrees +/- 0.3 degrees C with crystalloid cardioplegia [p less than .0001]; right ventricle: 18.3 degrees +/- 0.3 degrees C with blood cardioplegia and 15.1 degrees +/- 0.3 degrees C with crystalloid cardioplegia [p less than .0001]). Postoperatively, blood cardioplegia resulted in better LV performance (higher LV stroke work index at a similar LV end-diastolic volume index [EDVI]) (p = .01), better LV systolic function (similar systolic blood pressures at smaller LV end-systolic volume indexes [ESVI]), (p = .04), and improved LV diastolic function (lower left atrial pressures at similar LVEDVIs) (p = .03).(ABSTRACT TRUNCATED AT 250 WORDS)

Blood Pressure↗

Differential exercise performance on ventricular assist device support.

BACKGROUND: Ventricular assist devices (VADs) are approved for destination therapy because they improve survival in end-stage heart failure (HF). VADs are powered pneumatically or electrically. Pneumatic and electric left ventricular assist devices (LVADs) and biventricular assist devices (BiVADs) provide excellent hemodynamic support at rest, but differences in their effects on exercise tolerance are unclear. We sought to evaluate the effect of devices with varying operating parameters on exercise capacity. METHODS: Exercise physiology data obtained during maximal exercise with on-line gas-exchange analysis were collected for 38 consecutive VAD-implanted HF patients referred for exercise testing. RESULTS: Electric LVADs were implanted in 18 patients, and pneumatic LVADs in 10 patients. Percent of predicted peak exercise oxygen consumption (VO2%) was significantly greater in pneumatic LVAD patients (52.1 +/- 11.1% vs 38.2 +/- 11.3%, p < 0.05). The 10 patients implanted with a pneumatically powered LVAD were compared to 10 patients implanted with a pneumatically powered BiVAD. LVAD-supported patients had a higher VO2% (52.1 +/- 11.1% vs 36.5 +/- 17.7%, p < 0.05). CONCLUSIONS: HF patients supported with a pneumatic LVAD appear to have better exercise tolerance than those receiving an electric LVAD. Patients on LVAD support have better exercise tolerance than BiVAD-supported patients. This highlights the importance of right ventricular function to exercise tolerance in HF patients, and may have implications for future VAD design.

Adult↗

Analysis of T wave changes by activation recovery interval in patients with atrial septal defect.

We examined the distributions of the activation recovery interval (ARI), which is correlated with the local action potential duration (APD), to clarify the origin of the repolarization changes in ASD. The ECGs, QRST isointegral maps and ARI isochronal maps of 21 children with ASD from 3 to 5 years old in age were studied in comparison with 21 age-matched normal children. A conventional and 87 unipolar body surface ECG were simultaneously recorded. The ARIs were determined from the first derivatives of the ECG waveforms. Abnormal ST-T patterns were observed in 11 of 21 ASD, but only in two normal children. The QRST maps of a split positive area pattern were seen in 15 of ASD but none of the normal. In the ARI maps, all the normal children exhibited a short-ARI area on the left and a long-ARI area on the right side of the chest. In 19 of ASD, the ARI distribution revealed a leftward extension of the long-ARI area on the anterior chest, a relative shortening on the right anterior chest, and a localized prolonged ARI on the left anterior chest. The results suggest that right ventricular (RV) volume overload in ASD produces a localized prolongation of the APD on the RV epicardium.

Child, Preschool↗

Prognostic significance of right ventricular afterload stress detected by echocardiography in patients with clinically suspected pulmonary embolism.

OBJECTIVE: To investigate the prognostic value of echocardiographic findings in patients who present with symptoms suggestive of acute pulmonary embolism. DESIGN: 317 patients with clinically suspected pulmonary embolism were prospectively evaluated by echocardiography for the presence of right ventricular afterload stress and right heart or pulmonary artery thrombi. Objective confirmation of pulmonary embolism by lung scan or pulmonary angiography was obtained in 164 (52%). The presence of deep venous thrombosis was established in 90 of 158 patients (57%) who underwent phlebographic or Doppler sonographic studies. RESULTS: Right ventricular afterload stress was diagnosed in 87 patients (27%). Objective confirmation of pulmonary embolism and diagnosis of deep venous thrombosis was more common in patients with right ventricular afterload stress than in those without (83% v 40% and 46% v 22%, respectively; P < 0.001). This was also true for the detection of thrombi in the right heart and major pulmonary arteries (12 patients v 1 patient; P < 0.001) as well as for the in-hospital mortality from venous thromboembolism (13% v 0.9%; P < 0.001). One year mortality from pulmonary embolism was 13% in patients with right ventricular afterload stress at presentation compared with 1.3% in those without (P < 0.001). CONCLUSIONS: The presence of right ventricular afterload stress detected by echocardiography is a major determinant of short term prognosis in patients with clinically suspected acute pulmonary embolism.

Acute Disease↗

Norepinephrine can be useful for the treatment of right ventricular failure combined with acute pulmonary hypertension and systemic hypotension. A case report.

A 48-year-old woman who underwent emergency cardiac surgery for removal of a thrombus partially occluding the mitral valve, developed pulmonary hypertension right ventricular failure and systemic hypotension, in the immediate postoperative period, a clinical condition not well controlled by high doses of epinephrine and dobutamine. The addition of a continuous infusion of norepinephrine in incremental dosages, caused the rise in cardiac index accompanied before by the reduction in the pulmonary pressure and the stability in the systemic pressure, than by the further reduction in the pulmonary pressure and the increase in the systemic pressure. The conclusion is drawn that norepinephrine is useful in the treatment of right ventricular failure which follows a condition of acute pulmonary hypertension, because the improvement of cardiac performance established without adverse effects on the pulmonary pressures whose values on the contrary progressively declined.

Adrenergic alpha-Agonists↗

Severe pulmonary hypertension without right ventricular failure: the unique hearts of patients with Eisenmenger syndrome.

Why adults with the Eisenmenger syndrome fare so much better than other patients with severe pulmonary hypertension is not known, but may be related to unique hemodynamics found only in these patients and in normal fetuses. We used echocardiography to evaluate ventricular morphology and function in 80 subjects: 45 cyanotic adults and 5 cyanotic adolescents with Eisenmenger syndrome, 10 infants with nonrestrictive ventricular septal defect and left-to-right shunt flow (pre-Eisenmenger phase), and 20 fetuses with structurally normal hearts. Cross-sectional morphology of the hearts was the same in all 4 groups with a flat ventricular septum throughout the cardiac cycle and equal thickness of the right and left ventricular free walls (regression slope 0.98, r = 0.97, p <0.0001). This morphology was the same in patients independent of age, defect type, and ventricular function. Right ventricular fractional area change was slightly inferior to that of the left ventricle but normal in most patients with Eisenmenger syndrome (0.47 +/- 0.14 vs 0.51 +/- 0.13, p <0.01). Overall, there was a highly significant linear relation between right and left ventricular function (r = 0.81, p <0.0001). The hearts of patients with Eisenmenger syndrome are more like normal fetal hearts than normal adult hearts. Because of the unique cardiovascular hemodynamics, regression of right ventricular wall thickness does not occur and is likely the reason that patients with Eisenmenger syndrome fare so much better than other adults with severe pulmonary hypertension.

Adolescent↗

Venous return and right ventricular diastolic function in ARED flow fetuses.

The absence or reversal of end-diastolic flow in the umbilical artery (UA) is widely accepted as an ominous sign of fetal compromise. However, in these high-risk pregnancies, the significance of sequentially appearing changes of the venous return is poorly established. Doppler blood flow waveforms were longitudinally assessed in the inferior vena cava (IVC), ductus venosus (DV), umbilical vein (UV) and tricuspid valve, along with the UA, middle cerebral artery and descending aorta. Thirty-two serial Doppler studies were performed in six fetuses with intrauterine growth-retardation (IUGR). In the cases of absent or reversed UA endodiastolic flow, changes in the venous return were found following the same sequence: presence of dicrote pulsations in the UV, increase of retrograde flow in the IVC and reduction or inversion of DV velocity during atrial contraction. Transtricuspid flow patterns changed from normal to a restrictive pattern of ventricular filling. There were two intrauterine deaths and one early neonatal death. Our preliminary results suggest that the evaluation of the venous compartment of hemodynamically disturbed preterms fetuses along with right ventricular filling appears to be a useful model for investigating the physiopathology of fetal deterioration and, therefore, may yield indirect discriminatory signs of severe compromise.

Adult↗

[Arrhythmogenic right ventricle].

A 26-year-old patient with an irrelevant personal and family-history was treated on account of attacks of relapsing ventricular tachycardia and cardiac failure, mostly dextrolateral. After ruling out other possible causes of the disease--such as embolism of the lungs, ischaemic heart disease, out other possible causes of the disease--such as embolism of the lungs, ischaemic heart disease, Ebstein's anomaly, the diagnosis of an arrhythmogenic right ventricle was established. This disease is suggested by negative T waves in the thoracic leads V1-V3 during sinus rhythm and by the shape of the QRS complex which was the type found in block of the Tawara branch with an axis of + 100 degrees during attacks of ventricular tachycardia. Moreover, ventricular tachycardia of the same type was produced during electrocardiographic examination. On angiographic examination ARVD is suggested by marked trabeculization and impaired kinetics of the outflow tract of the right ventricle. In the prevention of relapses of ventricular tachycardia beta-blockers and Cordarone were successful when used simultaneously with cardiac stimulation type AAI applied on account of a confirmed sinoatrial block.

Adult↗