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Staged Fontan procedure for mitral atresia associated with severe tricuspid regurgitation, pulmonary hypertension, and pulmonary artery distortion.

Optimal initial palliation and a subsequent staged approach is mandatory for high-risk Fontan candidates. We describe the case of mitral atresia with severe tricuspid regurgitation and pulmonary hypertension successfully managed by repeated palliation from the neonatal period and 2-stage Fontan surgery. A 1-month-old boy diagnosed with mitral atresia and double-outlet right ventricle underwent pulmonary artery banding at 1 month of age, followed by repeated pulmonary artery banding accompanied by tricuspid annuloplasty and atrial septal defect enlargement at 6 months. Because of the presence of pulmonary artery distortion, right ventricular dysfunction, and borderline pulmonary vascular resistance, a hemi-Fontan procedure was conducted with extended pulmonary artery plasty when the boy was 3 years and 8 months old. Cardiac catheterization done 3 months after showed improvement in risk factors, and the final Fontan operation (total cavopulmonary connection) was successfully done in conjunction with repeated tricuspid annuloplasty when the boy was 4 years and 5 months old. The patient remains in excellent clinical condition at the last follow-up 5 years after the final Fontan procedure with sinus rhythm and good ventricular function.

Fontan Procedure↗

[Dynamic cardiomyoplasty].

Between 1965 and 1995 the incidence of heart failure has been constantly rising and the mortality from this disease has increased fivefold. The introduction of ACE-inhibitors and of adrenergic beta-blockers have resulted in major symptomatic improvements in patients with mild to moderate heart failure. For end-stage disease, heart transplantation offers by now the only therapeutic option and yields excellent results. The permanent implantation of left heart assist-devices is just gaining increasing importance. Yet, both methods also have inherent drawbacks and may not be available to all patients, so that new methods are constantly evaluated. Cardiomyoplasty was introduced into clinical practice in 1985 by Alain Carpentier and since then more than 700 patients have been operated worldwide. After dissection of the latissimus dorsi muscle it is wrapped around the heart in a clockwise fashion (Figure 1). Two sensing electrodes are placed on the anterior aspect of the right ventricle and two stimulation electrodes between the proximal branches of the thoracodorsal nerve (Medtronic SP 5548). The electrodes are then connected with a burststimulator (Cardiomyostimulator, Medtronic 4710) (Figure 2). During the first 2 weeks following the operation the muscle is not stimulated in order to allow for the healing process. Thereafter, a stimulation protocol with a programmed, staged increase of the stimulation frequency is started, to induce transformation of the skeletal muscle into a "fatigue resistant" tissue. After 3 months the muscle is stimulated with every second heart beat (2:1 mode) with full burstimpulses containing 6 single impulses per burst for a duration of 185 ms (Figure 3). Cardiomyoplasty was conceived for patients in NYHA III and severely impaired myocardial function, in whom drug treatment does not produce the expected benefits. The criteria for patient selection are strictly followed, since it has been shown in the past, that the preoperative condition of the patient is of specific importance for the postoperative outcome. Contraindications are NYHA IV, advanced right ventricular dysfunction, secondary pulmonary hypertension (> 600 dyn x s x cm-5), LV end-diastolic diameter > 70 mm und AV-valve incompetence > Grad II. Between July 1985 und October 1996 647 patients received a cardiomyoplasty with the Medtronic Cardiomyoplasty System and the results from 438 patients were analyzed from the "Worldwide Cardiomyoplasty Study Group". One and 2 years following the operation NYHA-class had improved by one class in 41.9% and 53.3%, respectively, and by 2 classes in 38.1% and 30.5%, respectively. In 16% and 15% no improvement was found (Figure 4). Prospective investigation of the quality of life by a score revealed a considerable improvement in the level of daily activities and social interaction. In contrast, two years after the operation, only a small, but significant increase in LV-EF from 22.9 +/- 8.1% to 25.8 +/- 9.7% (p < 0.05) was shown. Heart rate, maximal O2-consumption, total exercise time, cardiac index, stroke volume and stroke work index did not change. According to the results of a recent FDA-study, in-hospital mortality was 12% between 1991 and 1993, and was reduced during a second phase starting 1994 to < 3%. One, 2 and 3-year survival of 349 patients who were in NYHA-III prior to the operation was 69%, 56% und 47%, respectively. 43 patients who were operated in NYHA IV exhibited considerably worse survival with only 48% after 1 year and 30% after 2 years, respectively. In a subgroup of 103 patients with a statistically low operative risk, 1, 2 and 3-year survival was 77%, 71% und 61%, respectively (Figure 5). As a mechanism of action the skeletal muscle wrap exerts some active improvement of systolic wall motion of the heart/skeletal muscle complex. However, probably more important is an acute and chronically persisting shift of the pressure-volume relation to the left. This process results in a "reverse remodel

Cardiomyoplasty↗

Treatment of venous thromboembolism.

The combination of unfractionated heparin or low molecular weight heparin and oral anticoagulants is currently the treatment of choice for most patients with venous thromboembolism. Oral anticoagulants are started at the same time and heparin is discontinued after at least 5 days when the levels of the International Normalized Ratio reach the therapeutic range between 2.0 and 3.0. Low molecular weight heparin has potential advantages over heparin and is administered in subcutaneous weight-adjusted fixed doses without need for monitoring. This has made the home treatment of a large proportion of patients possible. Randomized clinical trials and several subsequent reports from clinical practice have demonstrated the efficacy and safety of this approach. The results of currently ongoing trials aimed to assess the efficacy and safety of newer compounds for the initial treatment of venous thromboembolism are expected. Oral direct thrombin inhibitors or selective factor-Xa inhibitors have the potential to become the treatment of choice in the next decade. The optimal duration of the secondary prophylaxis with oral anticoagulants is still a matter of debate. The rate of recurrence has been shown to be elevated, particularly in patients with idiopathic venous thromboembolism. A 3-month therapy is therefore currently recommended when a transient risk factor is identified, life-long treatment is recommended for patients with a second episode of venous thromboembolism. The presence of active cancer or a thrombophilic state may require long-term anticoagulation, although not all the congenital hypercoagulable states seem to carry the same level of risk. In all other cases, 6 months are recommended, but a long-term monitoring of the patients is advisable. The use of more aggressive strategies such as thrombolysis is limited to patients presenting with massive pulmonary embolism or signs of right ventricular dysfunction.

Anticoagulants↗

[Surgical treatment for massive pulmonary embolism].

Surgical embolectomy for massive pulmonary embolism (PE) has become a rare procedure. Often, it is viewed as a last-chance option for patients undergoing cardiopulmonary resuscitation after massive PE. Thus thrombolytic therapy has become the treatment of choice. However, a significant proportion of patients suffers from residual obstruction after thrombolytic therapy and faces the development of chronic pulmonary hypertension. Therefore, some centers have regained interest in surgical embolectomy after improved risk stratification and reported very good results. Perioperative survival rates up to 89% have been reported. This was accomplished by surgery on the ECC-(extracorporeal circulation-)supported, beating, normothermic heart and utilization of special instruments. These encouraging results have been confirmed by the authors and others in patients with stable systemic hemodynamics but moderate to severe right ventricular dysfunction. The more widespread use of surgical embolectomy seems warranted. A randomized, controlled trial is overdue to determine the benefits of this therapy in stable patients compared with thrombolytic therapy if "best-practice" therapy is to be achieved for the patients' benefit.

Embolectomy↗

What is evidence-based, what is new in medical therapy of acute heart failure?

Acute decompensated heart failure (ADHF) has become the leading cause of hospitalization in patients > 65 years of age. Traditional drug therapy for ADHF has remained unchanged for many years including morphine, diuretics, nitrates and inotropic agents in addition to oxygen supplementation and mechanical ventilatory support, if necessary. In the year 2005, the European Society of Cardiology published new guidelines for diagnosis and treatment of ADHF. These guidelines emphasize that ADHF is not a disease entity but a complex syndrome with various etiologies and several distinct clinical conditions as a result of systolic and/or diastolic left and/or right ventricular dysfunction. This review article describes the current role of traditional drugs for ADHF as well as the role of newer concepts including vasodilators like the recombinant human brain peptide nesiritide, endothelin antagonists or vasopressin antagonists and newer inotropic agents like the calcium sensitizer levosimendan.

Anti-Arrhythmia Agents↗

Hemodynamics in experimental gastric juice induced aspiration pneumonitis.

OBJECTIVE: To characterize hemodynamic changes during experimental aspiration pneumonitis, paying special attention to echocardiographic assessment. DESIGN AND SETTING: Animal study in a university-based research laboratory. SUBJECTS: Fourteen mechanically ventilated New Zealand white rabbits INTERVENTIONS: We instilled 1 ml/kg human gastric juice (mean pH: 4.1+0.2) intratracheally. Hemodynamic and respiratory parameters were measured every hour for 4 h, associated with a transthoracic echocardiography. MEASUREMENTS AND RESULTS: Lung injury occurred within 1 hour with a marked decrease in PaO(2)/FIO(2) and an increase in plateau pressure; after this initial drop the ratio remained stable throughout the experiment. Seven rabbits experienced only a mild to moderate alteration in lung oxygenation function as defined by a PaO(2)/FIO(2) ratio above 200 (group A), while the other seven developed a severe alteration with a ratio below 200 (group B). At the end of the experiment pH and cardiac output were lower in group B than in group A. Using a PaO(2)/FIO(2) threshold value of 150, pH, mean arterial pressure, and cardiac output were lower in the animals with the more severe hypoxemia. Neither left nor right ventricular dysfunction occurred during the experiment, and no animal experienced circulatory failure CONCLUSION: Experimental aspiration pneumonitis after intratracheal infusion of human gastric juice is characterized by a stable fall in PaO(2)/FIO(2). Animals with the most severe lung injury experienced a lower systemic arterial pressure, cardiac output, and metabolic acidosis without circulatory failure or cardiac dysfunction.

Animals↗

Monitoring the circulatory responses of shocked patients during fluid resuscitation in the emergency department.

OBJECTIVES: To assess the feasibility of constructing left ventricular response curves non-invasively during the fluid resuscitation of critically ill patients in the emergency department (ED) using a portable suprasternal Doppler ultrasound (PSSDU) device. DESIGN: Prospective case series. SETTING: Emergency department, Catholic University of Leuven, Belgium. PATIENTS: Shocked patients in the ED were diagnosed by predefined criteria. Only those thought to require standardised intravenous colloid challenges were observed i. e., sequential boluses of 3.5 ml/kg/10 min titrated against changes in stroke distance (Doppler surrogate for left ventricular stroke volume). RESULTS: A total of 50 shocked patients were studied. Stroke distance was measurable in 45 patients. 35 patients were fluid responders in terms of stroke distance. Group mean stroke distance increased during resuscitation (8.6 +/- 4.1 cm to 19.5 +/- 4.6 cm, P < 0.001) and then reached a plateau value (19.6 +/- 4.6 cm, P = 0.488). No response to fluid was seen in nine patients of which eight had severe sepsis. Alternative therapeutic approaches increased stroke distance for all of these patients. Evidence for right ventricular dysfunction was found as a cause for fluid non-response in the majority of patients with sepsis. CONCLUSIONS: Previous experimental work has shown that changes in central blood flow can be derived using the PSSDU device. This clinical feasibility study suggests that the PSSDU can help tailor haemodynamic therapy for an individual patient and give an early indication of treatment failure in the ED.

Adult↗

Evaluation of right ventricular performance long after the atrial switch operation for transposition of the great arteries using the Doppler Tei index.

Right ventricular (RV) dysfunction may occur in patients after the atrial switch operation for d-transposition of the great arteries (d-TGA) and can be an important complication. Noninvasive assessment of RV function using echocardiography is necessary for following up these patients. We evaluated RV function using the Doppler Tei index (RV Tei index). The RV Tei index measures the ratio of total time intervals in isovolumetric contraction and relaxation to the ejection time. The subjects consisted of 33 patients who had undergone the atrial switch operation for d-TGA (the atrial switch group) (mean age, 21 +/- 4 years), 10 patients with pulmonary artery stenosis after the arterial switch operation for d-TGA (the arterial switch group) (mean age, 17 +/- 1 years), and 10 patients with a normally structured heart as a control group (mean age, 26 +/- 7 years). The RV Tei index (0.65 +/- 0.16) was significantly higher in the atrial switch group than in the arterial switch group (0.29 +/- 0.07) and the control group (0.29 +/- 0.01) (p < 0.05). The fractional area change of the RV obtained from the four-chamber view in the atrial switch group showed a significant negative correlation with the RV Tei index (r = -0.58, p < 0.01). The RV Tei index increased with increasing severity of the NYHA functional class. In the moderate/severe tricuspid regurgitation group, it was significantly higher than in patients with none/trivial tricuspid regurgitation. We conclude that the RV Tei index is useful for evaluating RV junction long after the atrial switch operation for d-TGA.

Adolescent↗

Acute and Chronic Pulmonary Emboli.

Under most circumstances, the goal of treatment of pulmonary embolism is the prevention of recurrent embolic events, achieved through conventional anticoagulant therapy with unfractionated heparin or a low molecular weight heparin, followed by warfarin therapy for a minimum of 6 months. When acute pulmonary embolism is associated with significant right ventricular dysfunction or systemic hypotension, more aggressive intervention may be warranted. Under these circumstances, potential interventions include thrombolytic therapy (either systemic or catheter-directed), placement of an inferior vena caval filter, catheter-based embolectomy, or surgical embolectomy. Chronic thromboembolic pulmonary hypertension may develop in a small minority of patients who survive an acute, massive embolic event or who have suffered recurrent thromboembolic events. Due to the fixed nature of the pulmonary vascular obstruction, vasodilator therapy has proven far less effective in chronic thromboembolic disease than it has in primary pulmonary hypertension and other secondary forms of pulmonary hypertension. Correction of hypoxemia and volume overload and the prevention of recurrent embolic events are essential. Definitive therapy, however, requires surgical intervention to remove the chronic thromboembolic obstruction and to restore patency of the pulmonary vascular bed.

Journal Article↗

Right ventricular infarction with shock but without significant left ventricular infarction: a new clinical syndrome.

Right ventricular infarction has been described as concurrent with infarction involving the inferior (posterior) aspect of the left ventricular free wall and adjacent interventricular septum. Patients with right ventricular infarction typically show the ECG changes of inferior infarction in leads II, III, and aVF. This report describes two patients with right ventricular infarction but without changes in the QRS complex of the ECG, indicating an inferior infarct of the left ventricle. An autopsy-proven infarct of the right ventricular free wall with neither QRS nor other clinical evidence of left ventricular involvement was responsible for cardiogenic shock and death in one patient. This observation led to a review of a computerized data bank containing records of patients who had undergone cardiac catheterization to determine if there were other patients with clinical criteria suggesting right ventricular infarction without QRS changes of left ventricular infarction. One of the 167 patients with a history of a myocardial infarction also met the following clinical criteria: (1) transiently elevated total creatine kinase and creatine kinase myocardial band; (2) diffuse ST segment elevation without QRS changes indicative of left ventricular infarction on the ECG; (3) normal left ventricular function; (4) hemodynamic evidence of right ventricular dysfunction; and (5) cardiogenic shock.

Aged↗

Left ventricular systolic and diastolic function after two-stage anatomic correction of transposition of the great arteries.

Anatomic repair of transposition of the great arteries (TGA) has been developed because of concerns about right ventricular function after atrial repair by the Mustard or the Senning technique. This study assessed left ventricular systolic and diastolic function in three patients after two-stage anatomic repair. Two patients had a ventricular septal defect (one with coarctation), and the third patient had right ventricular dysfunction precluding atrial repair. All had pulmonary artery banding. The mean ages at the time of repair and catheterization were 2.75 and 4.9 years, respectively. The control group included 10 patients with insignificant or no cardiac disease. At cardiac catheterization the group with TGA had a higher mean end-diastolic volume index (110.9 +/- 4.74 ml/m2) compared to normal subjects (79.1 +/- 14.55; p less than 0.001), mean end-systolic volume index (37.3 +/- 3.69 vs 22.7 +/- 4.42; p less than 0.001), mass index (101.0 +/- 16.9 vs 68.2 +/- 12.34; p = 0.038), and stroke volume index (73.6 +/- 3.52 vs 56.5 +/- 12.1; p = 0.0027). The ejection fractions, end-diastolic and peak systolic pressures, and stresses were not different. There was no difference in the relationship between the mean rate-corrected velocity of circumferential fiber shortening and end-systolic stress for the group with TGA, but myocardial stiffness was markedly elevated (29.5 +/- 1.84 vs 10.8 +/- 2.20; p less than 0.001). Thus, this study found abnormalities of left ventricular size after two-stage anatomic repair of TGA in this group of patients with TGA.(ABSTRACT TRUNCATED AT 250 WORDS)

Cardiac Catheterization↗

Cardiac failure in coronary heart disease.

Cardiac failure, which used to be rare in coronary heart disease, is now its most common complication. Coronary heart disease can cause or appear as cardiac failure through one or more of 12 mechanisms: acute myocardial infarction, acute reversible ischemia, right ventricular dysfunction, cardiogenic shock, acute mitral regurgitation, ventricular septal perforation, cardiac free wall rupture, ischemic cardiomyopathy, ventricular aneurysm, coexisting diseases, iatrogenesis, and pseudoheart failure. An understanding of the responsible mechanism or mechanisms is essential not only for appropriate treatment but also for prognostication. Various therapeutic modalities, both medical and surgical, should be able to improve not only symptoms but also survival. Current efforts in the management of patients with cardiac failure as a result of coronary heart disease should be aimed at prevention, both primary and secondary.

Biomechanical Phenomena↗

The importance of pericardial constraint in experimental pulmonary embolism and volume loading.

To clarify the magnitude of the contribution of pericardial constraint to the hemodynamic deterioration that is observed during acute pulmonary embolism, hemodynamics and chamber dimensions (sonomicrometry) were measured during pulmonary embolization and subsequent volume loading in six anesthetized and instrumented open-chest, open-pericardium dogs. Embolization markedly increased peak right ventricular systolic pressure (38 +/- 5 mm Hg before embolism to 64 +/- 12 mm Hg after repeated embolization, p less than 0.05). However, right ventricular stroke volume decreased by only an insignificant amount (17 +/- 7 ml to 15 +/- 6 ml, p = not significant). Indices of left ventricular end-diastolic volume (left ventricular area = anteroposterior x septum-to-left ventricle free wall diameters) and stroke work (stroke work = area of the left ventricular pressure-area loop) were also similar before and after repeated embolization. Volume loading after repeated embolization resulted in increased right ventricular stroke volume (15 +/- 6 ml to 20 +/- 4 ml, p = 0.06), left ventricular area (3320 +/- 600 mm2 to 3470 +/- 580 mm2, p less than 0.05) and stroke work (261 +/- 158 mm Hg to 425 +/- 170 mm Hg x mm2, p less than 0.05). These results are in marked contrast to those in a previously reported study in a closed-chest and closed-pericardium model in which there was a decrease in left ventricular preload and systolic function after similar embolization-induced right ventricular pressure loading. Moreover, there was a further decrease in these parameters as a result of volume loading after embolism in the closed pericardium experiments. In conclusion, pericardial constraint contributes to hemodynamic deterioration during both acute right ventricular pressure loading and subsequent volume loading. The hemodynamic response to both interventions in the intact animal is determined not only by the degree of right ventricular dysfunction but also by the degree of direct ventricular interaction.

Animals↗

Myocardial imaging in the noninvasive evaluation of patients with suspected ischemic heart disease.

Three noninvasive radioactive tracer techniques for evaluating patients with ischemic heart disease are described: (1) myocaridal perfusion imaging, (2) acute infarct imaging, and (3) the gated blood pool scan. Myocardial perfusion imaging with tracers that distribute in the myocardium in relation to regional blood flow allows detection of patients with transmural and nontransmural infarction by the finding of decreased tracer concentration in the affected region of the myocardium. If these tracers are injected at the time of maximal stress to patients with significant coronary arterial stenosis but without infarction, areas of transient ischemia can be identified as zones of decreased tracer concentration not found when an examination is performed at rest. Acute infarct imaging with tracers that localize in acutely damaged tissue permits separation of patients with acute myocardial necrosis from those without infarction and those with more chronic damage. The gated blood pool scan permits assessment of left ventricular function and regional wall motion. The measurement of ventricular volumes, ejection fraction and regional wall motion adds significantly to the determination of hemodynamic variables in assessing patients with acute infarction. The technique also permits detection of right ventricular dysfunction. Performance of a combination of these radioactive tracer techniques is often advantageous, particularly in patients with suspected infarction. The techniques can establish whether infarction is present, whether it is acute, where the damage is located and how extensive it is; they can also provide a measure of the effect of this damage on left ventricular function.

Acute Disease↗

Mustard operation in the 1st month of life.

Since April 1976, nine infants less than 1 month of age underwent a Mustard intraatrial baffle procedure. All had transposition of the great arteries and were persistently symptomatic or hypoxic after the Rashkind balloon atrioseptostomy. Two had an associated large ventricular septal defect patched at the time of physiologic correction. The average age of the infants was 11.6 days (range 36 hours to 28 days). Weight averaged 3.4 kg (range 2.5 to 4.2). In all the Mustard procedure was performed using deep hypothermic circulatory arrest, averaging 71 minutes (range 48 to 88) at a mean core temperature of 13 degrees C. An average of 2.8 days of postoperative ventilatory assistance was required. One death occurred in a 28 day old male infant with an associated ventricular septal defect who, the morning after operation, had a sudden unresponsive cardiac standstill. The postoperative hospitalization period averaged 15 days (range 10 to 23). Follow-up evaluation has extended from 4 to 63 months (average 31). Six patients were restudied 2 to 21 months postoperatively. One had obstruction of the superior limb of the baffle, which had separated from the right atrial wall permitting a modest right to left shunt. She has had uneventful repair employing a unique modification of Senning's operation. Another child has asymptomatic partial obstruction of the superior limb of the baffle demonstrated angiographically. None has pulmonary venous obstruction and all have prevailing normal sinus rhythm. Significant right ventricular dysfunction has been demonstrated in one. Early correction in these persistently symptomatic or hypoxic neonates with transposition of the great arteries with or without a ventricular septal defect has obviated the need for palliative operations and produced gratifying early and late results.

Child, Preschool↗