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Combined atrial septostomy and oral sildenafil for severe right ventricular failure due to primary pulmonary hypertension.

Management of primary pulmonary hypertension is usually difficult because the disease is uncommon and the aetiology of the disease is not well understood. The disease is potentially lethal because it can lead to failure of the right ventricle, low cardiac output, and ensuing multiple organ failure. We report the successful treatment of a case of low-output syndrome due to primary pulmonary hypertension using combined drug therapy and atrial septostomy. Latest developments in the treatment of this disease are also discussed.

Adult↗

Use of myocardial performance index in pediatric patients with idiopathic pulmonary arterial hypertension.

BACKGROUND: The myocardial performance index (MPI) correlates with clinical status in adults with idiopathic pulmonary arterial (PA) hypertension (IPAH). This pediatric study used MPI to assess response to bosentan therapy. METHODS: The study included 12 children with IPAH and 12 healthy control subjects. MPI was correlated with catheterization data at initiation of bosentan and at a median follow-up of 9 months. Therapy responders were defined by a greater than 20% decrease in mean PA pressure. RESULTS: Right ventricular MPI for patients with IPAH was 0.64 +/- 0.30 versus 0.28 +/- 0.03 in control subjects (P < .01). It had a strong correlation with mean PA pressure (R = 0.94; P < .001). Right ventricular MPI decreased significantly in responders (range 20%-44%, mean 25%) with a 5% increase in nonresponders. CONCLUSIONS: Right ventricular MPI in pediatric IPAH correlates with mean PA pressure and response to therapy. This study suggests that this noninvasive Doppler index may be useful to follow up children with IPAH, particularly when tricuspid regurgitation data are insufficient.

Adolescent↗

The role of vasopressin in treating systemic inflammatory syndrome complicated by right ventricular failure.

Severe right ventricular failure complicated a postoperative systemic inflammatory response in a 33-year-old woman after surgical repair of congenital cardiac malformations. Volume loading, and high doses of catecholamines, failed to produce improvement, but treatment with vasopressin improved all haemodynamic parameters, and also allowed reduction of the other inotropes. After 10 days, the patient was discharged in stable condition from the intensive care unit.

Adult↗

[Guided anti-arrhythmia therapy by programmed ventricular stimulation. A case of arrhythmogenic dysplasia of the right ventricle].

Right ventricle dysplasia (RVD) is a rare clinical entity, described in young people, and a known cause of ventricular arrhythmias and sudden death. A genetic transmission has been suggested from the study of familial cases. The disease is characterized by a dilated right ventricle, cardiac infiltration with fatty tissue and focal fibrosis. Lymphoplasmocyte infiltrates are documented. The diagnosis is based on electrocardiography, echocardiography, magnetic resonance imaging and right ventricular endomyocardial biopsy. The authors report a case of a young woman with symptomatic ventricular tachycardia (VT) in whom the diagnosis of RVD was established. Programmed ventricular stimulation was used to reproduce the clinical VT and to test the efficacy of the anti-arrhythmic drug therapy. The diagnosis criteria and therapeutic options are discussed.

Adult↗

Demonstration of a His-Purkinje fatigue phenomenon with programmed stimulation of the right ventricular outflow tract.

This report describes the development of atrioventricular block by programmed stimulation of the right ventricular (RV) outflow tract in a patient with undiagnosed syncope. Burst pacing from the RV apex and outflow tract and programmed stimulation from the RV apex were unsuccessful. The observations were consistent with the fatigue phenomenon of the His-Purkinje system and illustrate the importance of an appropriate stimulation protocol in the electrophysiological evaluation of syncope.

Aged↗

[Arrhythmogenic right ventricular cardiomyopathy. Etiology, diagnosis and therapy].

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is characterized by regional atrophy of right ventricular myocardium and subsequent replacement by fatty and fibrous tissue. The disease manifests in young adulthood with a predominance of males. Hallmarks of ARVC are ventricular tachyarrhythmias of left bundle branch block pattern which frequently occur during exercise. However, sudden death may also be the first manifestation of the disease. Characteristic findings are repolarization abnormalities and QRS prolongation in the right precordial leads of the surface ECG and regional abnormalities of right ventricular structure and wall motion. Left ventricular involvement may occur in later stages of the disease but rarely leads to progressive biventricular heart failure. Therapeutic efforts are mainly directed to the treatment of ventricular tachyarrhythmias and the prevention of sudden death. A tailored treatment strategy including antiarrhythmic drug therapy, catheter ablation and implantation of cardioverter-defibrillators may be used to improve the long-term prognosis of patients with ARVC.

Anti-Arrhythmia Agents↗

Usefulness of magnetic resonance imaging dobutamine stress in asymptomatic and minimally symptomatic patients with decreased cardiac reserve from congenital heart disease (complete and corrected transposition of the great arteries and subpulmonic obstruction).

We explored the effect of dobutamine stress and its possible clinical implications in different groups of asymptomatic patients with chronic right ventricular (RV) pressure overload due to congenital heart disease. Forty-seven asymptomatic and minimally symptomatic patients with chronic RV pressure overload were studied: 24 patients with systemic right ventricles (16 surgically corrected transposition of the great arteries (TGA) (Mustard or Senning), 8 congenitally corrected TGA), 23 patients with chronic pressure overloaded subpulmonic right ventricles, and 11 age- and sex-matched healthy volunteers. Magnetic resonance imaging (MRI) was performed both at baseline and during dobutamine stress to determine RV volumes and ejection fraction. At baseline, RV ejection fraction in patients with surgically corrected TGA was significantly lower than in controls (58 +/- 10% vs 70 +/- 8%, p = 0.02). During dobutamine stress, RV ejection fraction increased significantly in controls and patient groups except for patients with pressure overloaded subpulmonic right ventricles. RV stroke volume increased in controls (21 +/- 21%, p = 0.008); RV stroke volume remained unchanged in patients with congenitally corrected TGA and surgically corrected TGA (2 +/- 17%, p = NS; -8 +/- 29%, p = NS). A significant RV stroke volume decrease was observed in patients with subpulmonic right ventricles (-15 +/- 16%, p = 0.0002). The changes in RV stroke volume were accompanied by a significant decrease in RV end-diastolic volume (-13 +/- 14%, p = 0.001) in patients with subpulmonic right ventricles and in patients with surgically corrected TGA (-23 +/- 16%, p = 0.0001). In controls and in patients with congenitally corrected TGA there was no change in RV end-diastolic volume (3 +/- 15%, p = NS; -5 +/- 11%, p = NS). There is a clear heterogeneity in response to MRI dobutamine stress between different groups of patients with chronic RV pressure overload. Our data suggest impaired filling in surgically corrected TGA and decreased contractility in patients with chronic pressure overloaded subpulmonic right ventricles. Dobutamine stress MRI may facilitate follow-up of RV (dys)function in patients with chronic RV pressure overload due to congenital heart disease.

Adult↗

Double chambered right ventricle: delineation by multiplane transoesophageal echocardiography.

Three consecutive patients (median age 5 years; median weight 15 kg) with double chambered right ventricle (DCRV) were studied by multiplane transoesophageal echocardiography (TOE), using a specially designed paediatric probe. Using a 30 degrees angulation from the longitudinal plane, the body and outflow tract of the right ventricle could be imaged with minimal foreshortening, allowing detailed description of the level and nature of obstruction. In all cases, angiography and subsequent surgical inspection confirmed the TOE findings. Multiplane TOE should avoid the necessity for angiography in the preoperative assessment of this unusual lesion.

Cardiac Catheterization↗

Undersensing of the tiny QRS complexes that emerged after the isolation of the right ventricle in a patient with ventricular tachycardia.

This article describes a patient who underwent right ventricular disconnection for medically refractory ventricular tachycardia associated with arrhythmogenic right ventricular dysplasia. After the operation there was no ventricular tachycardia recurrence. Two years after the operation, he received a permanent VVI pacemaker for the symptomatic second-degree AV block. Sensing function of the pacemaker was normal for the normal QRS complexes, but the tiny QRS complexes that appeared after the arrhythmia surgery were not sensed by the pacemaker and therefore caused no problem.

Adult↗

Ascites and other extracardiac manifestations associated with right atrial myxoma: a case report.

Right atrial myxomas are rare intracardiac tumors that often pose difficulties in diagnosis. Right ventricular failure and ascites ensuing from tricuspid valve orifice obstruction are potentially dangerous complications. Early diagnosis of cardiac myxoma is important since surgical treatment leads to disappearance of all symptoms with a low rate of recurrence and good long-term survival. Nonspecific extracardiac symptoms, signs, complications, and laboratory findings may be the initial manifestations contributing to misdiagnosis of these rare but totally treatable atrial tumors.

Heart Atria↗

Right ventricular involvement in Takotsubo cardiomyopathy.

AIMS: The aim of our study is to assess the incidence and clinical significance of right ventricular (RV) involvement in Takotsubo cardiomyopathy (TTC). METHODS AND RESULTS: Between February 2002 and December 2005, 47 patients with TTC underwent cardiovascular magnetic resonance (CMR) at our institutions. 13 patients with delayed initial CMR were excluded. In the remaining 34 patients (32 women), RV wall motion abnormalities (WMAs) were present in nine (26%). Left ventricular ejection fraction (LVEF) was significantly lower in patients with RV involvement (40 +/- 6 vs. 48 +/- 10%, P = 0.04). The most frequently affected RV segments were the apico-lateral (89%), the antero-lateral (67%), and the inferior segment (67%). All RV WMA improved or disappeared in eight of nine patients who underwent a follow-up CMR study. Pleural effusion was more common in patients with RV involvement (67 vs. 8%, P < 0.001) and was predictive of RV dysfunction (sensitivity 67% and specificity 92%). Significant or bilateral pleural effusions were seen exclusively in patients with RV involvement. CONCLUSION: RV involvement is common in TTC and seems to be associated with a more severe impairment in LV systolic function. It may be suspected by the presence of pleural effusion.

Aged↗

Beyond the basics: right vs. left heart failure.

Heart failure is a pathophysiologic cascade that, if improperly identified and managed, may lead to death. If heart failure is identified early enough, development of irreversible end-stage heart failure may be avoided. The goal of the prehospital care provider in managing heart failure is directed at maintenance of adequate cellular oxygenation, reduction of preload and afterload to improve forward blood flow, and, ultimately, improve cardiac output.

Education, Continuing↗

Tricuspid valve repair in hypoplastic left heart syndrome.

OBJECTIVES: Currently, the survival for the Norwood procedure for hypoplastic left heart syndrome is approximately 90% in selected centers. However, the development of tricuspid regurgitation remains a significant obstacle to successful staged repair in a subset of these patients. The results of tricuspid valve repair in this challenging patient population remain largely unknown. METHODS: Twenty-eight patients with significant (3-4+) tricuspid regurgitation after the Norwood procedure required tricuspid valve repair from August 1995 through December 2002. The clinical and Doppler-echocardiographic data were reviewed to determine the efficacy of repair and patient outcome. RESULTS: Follow-up was 96% complete (27/28). Patients were divided into 2 groups on the basis of tricuspid regurgitation at late follow-up: those with a successful late outcome (0-2+) and those with a poor outcome (3-4+). There were 17 (63%) patients with a successful result and 10 (37%) with an adverse outcome. Age, weight, follow-up duration, valve anatomy, and stage of palliation were not significantly different between groups. Early postoperative 0 to 2+ regurgitation was associated with a durable result (P =.012) and preserved ventricular function (P =.04). Need for repair other than a partial annuloplasty was predictive of a poor outcome (P =.04). Overall survival was 67% (18/27). Survival was 94% (16/17) for patients with a successful late result versus 20% (2/10) for those with a poor outcome (P =.0002). CONCLUSIONS: Tricuspid valve repair can be accomplished in this challenging patient population with excellent results. Successful tricuspid valve repair is predictive of continued good valve function and preserved right ventricular function. Successful valve repair at late follow-up predicts excellent late survival.

Child↗

Acute right ventricular failure during aortic valvular operation due to mechanical problem in the right coronary artery.

BACKGROUND: When right ventricular failure occurs during aortic valvular operation, inadequate myocardial protection may be the culprit. However, other causes of right ventricular failure should be considered, such as air or particulate matter embolization. Right ventricular failure unexpectedly occurred during an aortic valvular operation, and the cause was found to be a mechanical problem with the right coronary ostium. METHODS: A mechanical problem with the right coronary artery (RCA) occurred in 9 patients undergoing aortic valvular procedures, which included isolated aortic valve replacement (4 patients), aortic valve replacement and coronary artery bypass (1 patient), Bentall procedure (2 patients), aortic valve reconstruction (1 patient), and double valve replacement and coronary artery bypass (1 patient). Although myocardial protection was considered to be the cause, a mechanical problem was subsequently identified in the RCA, leading that artery to be bypassed with a segment of saphenous vein. RESULTS: The 1 patient in whom the condition was not recognized at time of aortic valve operation died; at autopsy, a damaged and occluded right ostium was confirmed. The other 8 patients who had the RCA bypassed survived. CONCLUSIONS: We conclude that when right ventricular failure unexpectedly occurs during an aortic valvular operation and does not improve with reperfusion, a mechanical problem in the RCA should be considered. In this situation we recommend that the RCA be bypassed with a segment of saphenous vein graft.

Acute Disease↗

[Late complications following early childhood atrial switch operations for d-transposition of the great arteries. Incidence, diagnosis and therapy].

BACKGROUND AND OBJECTIVE: The atrial switch operation in patients with transposition of the great arteries have been replaced by the arterial switch operation in the neonatal period. However, an increasing number of atrial switch survivors has now reached adulthood. This study was aimed at evaluating the incidence and patterns of typical late complications in order to describe diagnostic and therapeutic options. PATIENTS AND METHODS: We retrospectively analysed the data of 40 adolescent and adult patients, who had an atrial switch operation a mean of 17,9 years ago (23 Senning, 17 Mustard operations). The mean age of the patients is 19,3 years with 40 % females. The follow up time in our institution was 4,7 years. RESULTS: The most frequent complications were cardiac rhythm disturbances (47,5 % loss of sinus rhythm, 22,5 % intermittent atrial flutter, 22,5 % junctional rhythm, 7,5 % paroxysmal supraventricular tachycardia, 17,5 % sick sinus syndrome, 5 % premature ventricular contractions), severe tricuspid regurgitation (15 %), severe right ventricular failure (5 %), baffle leaks (27,5 %), baffle obstructions (17,5 %), and pulmonary artery hypertension. CONCLUSION: The data demonstrate that adult patients who underwent atrial switch operation in early childhood need long term follow-up to allow early recognition and treatment of late complications, even in asymptomatic patients.

Adolescent↗

Syncopal sustained ventricular tachycardia in a patient with right ventricular dysplasia.

A 41-year-old man was hospitalized for syncopal sustained ventricular tachycardia with left bundle branch morphology. Diagnostic screening confirmed a right ventricular dysplasia: fibrofatty replacement of myocardium on endomyocardial biopsy and severe dilation of right ventricle with no left ventricular impairment was documented. His bundle recording showed an abnormally long HV interval, and programmed ventricular stimulation induced high-rate sustained ventricular tachycardia with left bundle branch block morphology associated with reduced systolic blood pressure and dizziness. Right ventricular burst pacing proved to be effective in restoring sinus rhythm. A single lead pectoral cardioverter-defibrillator was implanted and programmed for VVI and antitachycardia pacing, as first ventricular tachycardia therapeutic intervention. During 6-month follow-up, 1 asymptomatic ventricular tachycardia recurrence terminated by antitachycardia pacing was reported by the device. The possible role of the other therapeutic options such as drugs, ablation, and surgery for this specific case is also discussed.

Adult↗

Echocardiographic abnormalities in sickle cell disease.

Echocardiographic abnormalities in patients with sickle cell disease (SCD) were determined, and pulmonary arterial systolic pressure (PASP) was estimated. Clinical data and echocardiograms of 38 adult hospitalized patients with SCD at two tertiary care hospitals were reviewed. Fisher's exact test was performed to determine correlation between pulmonary hypertension and various clinical variables. Pulmonary hypertension was the most common abnormality identified in 22 (58%) patients. The estimated mean PASP was 37.5 +/- 10.9 mmHg. Older age and prior history of acute chest syndrome were significantly correlated with an increased prevalence of pulmonary hypertension (P < 0.05). Patients with hemoglobin levels <8 g/dL had PASP 43.2 +/- 0.5 compared to a mean PASP of 33.3 +/- 6.0 in patients with hemoglobin > or =8 g/dL (P = 0.01). Eight (21%) patients had evidence of a hyperdynamic left ventricle. Left heart abnormalities included dilated atrium in 14 (37%), dilated ventricle in 5 (13%), ventricle hypertrophy in 5 (13%), and ventricle dysfunction in 3 (9%) patients. Right heart abnormalities included dilated atrium in 9 (24%), dilated ventricle in 6 (16%), and ventricle dysfunction in 3 (9%) patients. Despite an increased incidence of abnormal flow across the valves on Doppler analysis, no patient had structurally abnormal valves. A majority of patients with SCD had evidence of pulmonary hypertension, which correlated with older age and history of acute chest syndrome. Other structural and functional echocardiographic abnormalities were less common.

Adult↗

Cardiovascular limitations in chronic pulmonary disease.

Chronic lung disease has significant impact on cardiovascular function. Much of this effect is because of increased right ventricular afterload caused by increased pulmonary vascular resistance resulting from structural changes in the pulmonary circulation, and because of hypoxic pulmonary vasoconstriction. In the case of chronic obstructive diseases, there may be additional increases in afterload resulting from dynamic hyperinflation. These processes can lead to structural changes in the heart (cor pulmonale), including right ventricular dilatation and hypertrophy, to maintain right ventricular output. In most ambulatory patients with chronic obstructive disease, it appears that cardiac output may be maintained at levels that are similar to normal both at rest and during exercise, with no consistent improvement in maximal exercise function afforded by interventions that increase blood flow. In contrast, diseases characterized by fibrosis or infiltration of the lung parenchyma may be associated with a disproportionate increase in pulmonary vascular resistance and more pronounced cardiovascular impairment, particularly with exercise.

Cardiovascular System↗