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Cardiac sarcoidosis mimicking right ventricular dysplasia.

A 59-year-old woman with skin sarcoidosis was admitted to hospital for assessment of complete atrioventricular block. Cross-sectional echocardiography showed that the apical free wall of the right ventricle was thin and dyskinetic with dilation of the right ventricle. Thallium-201 myocardial imaging revealed a normal distribution. Both gallium-67 and technetium-99m pyrophosphate scintigraphy revealed no abnormal uptake in the myocardium. Right ventriculography showed chamber dilation and dyskinesis of the apical free wall, whereas left ventriculography showed normokinesis, mimicking right ventricular dysplasia. Cardiac sarcoidosis was diagnosed on examination of an endomyocardial biopsy specimen from the right ventricle. A permanent pacemaker was implanted to manage the complete atrioventricular block. After steroid treatment, electrocardiography showed first-degree atrioventricular block and echocardiography revealed an improvement in the right ventricular chamber dilation. Reports of cardiac sarcoidosis mimicking right ventricular dysplasia are extremely rare and as this case shows, right ventricular involvement may be one of its manifestations.

Arrhythmogenic Right Ventricular Dysplasia↗

Late complications and quality of life after atrial correction of transposition of the great arteries in 12 to 18 year follow-up.

BACKGROUND: Most adolescents and young adults born with transposition of the great arteries and survivors of Mustard operation are alive today and are leading ordinary lives. How far is the quality of their life influenced by residual findings? METHODS: We have analysed the oldest group of 177 children operated on with the Mustard procedure during 1979-1984 from our total experience of 609 corrections of transposition of the great arteries until 1996. In the long-term follow-up 12 to 18 years after Mustard operation the clinical investigation, 24 hours ECG Holter monitoring, 2D and colour flow Doppler echocardiography, radionuclide angiocardiography and exercise testing were performed. RESULTS: Basic sinus rhythm had 61% of 137 survivors, sinus node dysfunction had 51%. All but 4 patients needed no antiarrhythmic therapy. There were 10 sudden deaths. Severe tricuspid valve regurgitation developed in 15.9% of children, and decreased to 6% after intensive medical treatment. The right ventricular systolic dysfunction was found on radionuclide ventriculography in 8% of children after surgery. The left ventricular ejection fraction was subnormal in 10% and left ventricular diastolic function expressed by rapid filling fraction was subnormal in 80% of patients and may cause inability to increase cardiac output at exercise The obstruction of caval veins was found in 9%. There were neurological complications in 13.3% (10% already preoperatively) but severe brain damage was found in 4.4% only. CONCLUSIONS: Exercise tolerance with the maximum oxygen consumption over 30 ml/kg/min in 96% of investigated children suggests the good ability and full efficiency for recreation sports. Most survivors (84%) are reported to lead ordinary lives and function in NYHA class I.

Adolescent↗

Early results after transatrial/transpulmonary repair of tetralogy of Fallot.

OBJECTIVES: Right ventricular (RV) dysfunction is a significant cause of morbidity and mortality after surgical correction of tetralogy of Fallot (TOF). Transatrial/transpulmonary repair avoids a ventriculotomy (in contrast to the transventricular approach) emphasizing maximal preservation of RV structure and function. We have adopted this technique as less traumatic for the right ventricle. This study evaluates the early surgical results of our approach. METHODS: Between September 1997 and July 2001, 110 consecutive patients with TOF were referred to our unit for surgical therapy. Of these, 14 were unsuitable for repair and underwent aortopulmonary shunting+/-pulmonary artery patching. In the remaining 96 patients (median age 1.4 years), complete transatrial/transpulmonary repair was performed. Previously placed shunts (ten patients) were taken down and any secondary stenoses or branch pulmonary artery distortion repaired. In all cases, subpulmonary resection and ventricular septal defect (VSD) closure were accomplished transatrially. Whenever pulmonary valvotomy and valve ring widening were necessary, it was achieved through a pulmonary arteriotomy. In 84 patients the main pulmonary artery was augmented with an autologous pericardial patch, and in 23 the patch was extended to pulmonary artery branch(es). A limited (<1cm ) or extended (>1cm, but <or=length of RV infundibulum) transannular incision was necessary in 59 and 18 patients, respectively, in order to achieve an adequate residual RV outflow tract diameter. A monocusp autologous pericardial valve was placed in 13 patients. RESULTS: There was no death in this series. No patient required permanent pacemaker. In one case, early reoperation for residual RV outflow tract obstruction was needed. Median ICU and hospital stay were 3.5 and 10 days, respectively. At median follow up of 26 (mean 25+/-12) months, all patients are asymptomatic, with no significant residual lesion. CONCLUSIONS: Transatrial/transpulmonary repair of TOF is associated with remarkably low morbidity and mortality in our early experience.

Cardiopulmonary Bypass↗

Increased calcium deposits and decreased Ca2+-ATPase in right ventricular myocardium of ascitic broiler chickens.

Right ventricular hypertrophy and failure is an important step in the development of ascites syndrome (AS) in broiler chickens. Cytoplasmic calcium concentration is a major regulator of cardiac contractile function and various physiological processes in cardiac muscle cells. The purpose of this study was to measure the right ventricular pressure and investigate the precise ultrastructural location of Ca(2+) and Ca(2+)-ATPase in the right ventricular myocardium of chickens with AS induced by low ambient temperature. The results showed that the right ventricular diastolic pressure of ascitic broilers was significantly higher than that of control broilers (P < 0.01), and the maximum change ratio of right intraventricular pressure (RV +/- dp/dt(max)) of ascitic broilers was significantly lower than that of the controls (P < 0.01). Extensively increased calcium deposits were observed in the right ventricular myocardium of ascitic broilers, whereas in the age-matched control broilers, calcium deposits were much less. The Ca(2+)-ATPase reactive products were obviously found on the sarcoplasmic reticulum and mitochondrial membrane of the control right ventricular myocardium, but rarely observed in the ascitic broilers. The data suggest that in ascitic broilers there is the right ventricular diastolic dysfunction, in which the overload of intracellular calcium and the decreased Ca(2+)-ATPase activity might be the important factors.

Animals↗

High-output heart failure as a cause of pulmonary hypertension.

Two cases of high-output heart failure associated with pulmonary hypertension are presented. In a 32-year-old man with hyperthyroidism, pulmonary hypertension subsided after antithyroid therapy. Increased pulmonary blood flow and elevated left ventricular end-diastolic pressure were suspected as the causes of pulmonary hypertension. In a 51-year-old man with cardiac beriberi, the pulmonary hypertension improved after thiamine administration. We suspect that increased pulmonary blood flow, elevated left ventricular end-diastolic pressure, and probably pulmonary vasoconstriction were the causes of pulmonary hypertension. Thus, high-output heart failure should be considered as a possible cause of pulmonary hypertension and right ventricular failure.

Adult↗

[Doppler tissue imaging of the myocardium and right ventricle].

Doppler tissue imaging is a technique of analysis of myocardial wall motion. It may easily be used for the right ventricular walls, especially in the apical 4-chamber view, from which the velocities of the RV free wall can be measured from the tricuspid annulus to the apex. The regional velocities of the right ventricle in its long axis and the excursion of the tricuspid annulus are higher than those of the left ventricle but the velocities of circumferential fibre shortening are lower. Moreover, the time interval between the end of the Sm wave and the onset of the Em wave (equivalent to the isovolumic relaxation time) is almost virtual in the right ventricle. The parameters of deformation (strain rate, strain) are also higher in the right than in the left ventricle and are variably distributed. Several studies have examined the clinical value of Doppler tissue imaging of the right ventricle. In cardiac failure, a significant correlation between the systolic velocity of the tricuspid annulus displacement and right ventricular ejection fraction has been reported. The ratio of tricuspid E wave velocity to the tricuspid annulus B wave allow assessment of right ventricular filling pressures. The measurement of tricuspid annulus velocities could be diagnostic of right ventricular extension of an inferior wall infarct. The recording of a "rebound" positive wave just after the E wave in the RV and LV free walls (and in the interventricular septum) is a diagnostic sign of constrictive pericarditis. Doppler tissue imaging is therefore a promising technique for evaluating right ventricular function.

Diagnosis, Differential↗

Utility of ST segment depression in lead AVL in the diagnosis of right ventricular infarction.

We performed twelve lead electrocardiograms(ECG) and right precordial leads on twenty-two consecutive patients with first inferior wall acute myocardial infarction (MI) diagnosed by classical Q waves and elevation of cardiac enzymes. The presence of right ventricular MI was established by either technetium 99 (TC-99) pyrophosphate scanning or 2-dimensional (2-D) echocardiography by observers unrelated to the study and not aware of the electrocardiographic findings. In patients with established right ventricular MI (n = 15/22), ST segment elevation > or = 0.1 mV in lead V4R placed in the right precordial lead position had 91% (10/11) sensitivity in diagnosing right ventricular MI. ST segment depression in lead AVL > or = 0.1 mV had 100% sensitivity in diagnosing right ventricular MI (15/15). In the seven patients without right ventricular infarction, the absence of ST segment elevation in lead V4R had 100% specificity (7/7), while the absence of ST segment depression in lead AVL had 57% specificity (4/7) in excluding right ventricular infarction. The data indicate that in the presence of inferior wall MI, ST segment depression in lead AVL is a sensitive, but not very specific sign of right ventricular MI. Therefore, its presence in patients with inferior wall MI should lead to further investigations to diagnose or exclude right ventricular MI.

Adult↗

Disintegration of the 'waterfall phenomenon' in the inferior vena cava due to right heart failure.

The concept of the waterfall phenomenon in Zone 2 in the pulmonary vasculature is well known from West's lung model. It is believed that the flow through this zone is determined by the pressure difference between the pulmonary artery and alveoli, and the left atrial pressure is not transmissible to the alveolar capillaries. However, it is impossible to see whether alveolar capillaries are really displaying the waterfall phenomenon or not. In this review, the interrelation between the flow and geometry of the alveolar capillaries in the waterfall phenomenon is analyzed based on physiological studies using a model system and isolated lung lobe experiments. Further, extending the concept to the analysis of ventilatory changes of the inferior vena cava (IVC) configuration, it is ascertained that the waterfall phenomenon normally occurs in the IVC during inspiration just before it enters the thorax and the waterfall phenomenon in the IVC disintegrates with elevation of the central venous pressure. Because these configurations of the IVC in normal and abnormal conditions are visible with ultrasonography, the technique is very useful as a noninvasive approach to diagnose right heart failure.

Blood Flow Velocity↗