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PubMedDate not supplied

Right ventricular function in congenital heart disease: pressure and volume overload lesions.

The right ventricle is often subject to both pressure and volume overload in congenital heart disease. Evaluating right ventricular function in both the native lesion and after surgery in light of these loading conditions, presents a unique challenge for investigators studying these misshapen hearts. The purpose of this article is to briefly delineate what is generally known about right ventricular function in congenital heart disease and to touch on some noninvasive imaging modalities which have helped shed some light on this matter.

Animals↗
PubMed1996

Physiology of univentricular versus biventricular support.

Right ventricular failure unresponsive to pharmacologic treatment occurs in approximately 20% to 30% of patients supported with a left ventricular assist device (LVAD). The effect of the assistance on right ventricular function is highly controversial. Increased venous return produced by an LVAD can affect right ventricular function by increasing preload. On the other hand, an LVAD can improve the filling of the right ventricle by unloading the left ventricle, reducing its chamber size and shifting the septum back to the left. Right ventricular function is highly afterload dependent, the ventricular function depending on the pulmonary vascular resistance. With a normal pulmonary vascular bed, the LVAD can improve right ventricular function by reducing right ventricular afterload. If there is a fixed high pulmonary pressure, however, the LVAD can increase right ventricular afterload and volume. We conclude that the right ventricle is dispensable if the pulmonary vascular bed is normal.

Cardiac Output, Low↗
PubMed1992

Fatal pulmonary hypertension in a patient with mixed connective tissue disease: report of an autopsy case.

A 34-yr-old woman with mixed connective tissue disease (MCTD) who died of heart failure due to pulmonary hypertension (PH) is reported. She showed various symptoms and signs over the previous 8 yr, including lymphadenopathy, Raynaud's phenomenon, polyarthralgia, sclerodactylia, esophageal disturbance, PH, Sjögren's syndrome and renal tubular acidosis. Autopsy findings revealed severe intimal proliferation with marked luminal narrowing of the small arteries in the lung. Echocardiographic and mechanocardiographic methods were very useful for assessment of the degree of right ventricular dysfunction associated with PH on the early stage of MCTD.

Adult↗
PubMed2003

The effect of pulmonary circulation hemodynamics on right ventricular unloading via the bidirectional Glenn shunt: implications for congenitally corrected transposition repair.

The bidirectional Glenn shunt has been successfully applied as an adjunct to ventricular septal defect closure and pulmonary valvulotomy to treat congenitally corrected transposition of the great arteries (ccTGA). The purpose of this study was to examine the volume and pressure unloading effects of the bidirectional Glenn shunt on the hypertrophied pulmonary ventricle in a canine model of ccTGA. Five beagles underwent survival surgery to band the pulmonary artery. Three months later, a polytetrafluoroethylene graft was anastomosed to the superior vena cava and right pulmonary artery. The graft or superior vena cava was clamped to create the normal or bidirectional Glenn circulation, and hemodynamic data were recorded. The bidirectional Glenn shunt significantly reduced right ventricular volume loading and stroke work. Dogs with normal pre-bidirectional Glenn cardiac outputs had greatly reduced right ventricular volumes and pressures with the bidirectional Glenn shunt. Dogs with pre-bidirectional Glenn right ventricular dysfunction had moderate volume but no pressure decreases with the bidirectional Glenn shunt owing to improved left ventricular output. In these dogs it is likely that the decreased level of pressure and volume unloading is because of a concomitant improvement in left ventricular output post-bidirectional Glenn shunt placement. The bidirectional Glenn shunt is effective at unloading the right ventricle in a canine model of ccTGA.

Animals↗
PubMed2006

[Implantation of mechanical pulmonary and tricuspid valve prostheses at a distance from complete repair of Tetralogy of Fallot].

The authors report the case of a 39 years old woman operated for tetralogy of Fallot at the age of 6. Multiple complications due to postoperative atrioventricular block and a poor surgical result on the pulmonary outflow tract led to several reoperations. Right ventricular dysfunction with pulmonary regurgitation and mitral tricuspid valve disease in a context of endocarditis on the pacing catheter led to double pulmonary and tricuspid valve replacement with mechanical prostheses. The outcome at follow-up at 3 years is good. To the authors' knowledge, this is the first reported case of double mechanical valve replacement of the right heart after complete repair of tetralogy of Fallot.

Adult↗
PubMed1989

Pulmonary allograft conduit repair of tetralogy of Fallot. An alternative to transannular patch repair.

Cryopreserved allograft valves and valved conduits have been used in 122 patients during the past 3 years for surgical repair of congenital heart disease. In 55 of these patients, the right ventricular outflow tract was reconstructed with a pulmonary allograft conduit. Although most children requiring a conduit had complex lesions, 12 patients with tetralogy of Fallot without pulmonary atresia or absent valve syndrome have been treated in this manner since April 1985. Patients were selected for conduit therapy because of congenital or iatrogenic pulmonary artery problems (nine patients had 12 prior shunts) or increased pulmonary vascular resistance. Ages ranged from 7 months to 6 years (mean 3.1 years) and weights from 7.4 to 18.5 kg (mean 12.2 kg). Pulmonary artery conduit size ranged from 16 to 24 mm internal diameter (mean 22 mm). Distal pulmonary artery reconstruction beyond the bifurcation was required in nine patients and the proximal connection was completed with a hood-shaped patch. One operative death (8.3%) occurred at 20 days as a result of severe right ventricular dysfunction. Left pulmonary artery thrombosis was discovered postmortem. Eleven survivors observed for 3 to 37 months (mean 17 months) are without cardiac symptoms. A chronologically overlapping group of tetralogy of Fallot repairs accomplished with a transannular patch was also reviewed. Twenty-five patients aged 2 months to 4 years (mean 1.7 years) and weighing 3.6 to 14.8 kg (mean 9.2 kg) underwent this procedure from April 1983 to January 1987 (seven patients had one prior shunt each). The mortality rate in this group was 28% (six operative deaths and one late death). Of 18 survivors observed from 20 to 60 months (mean 41.4 months), five (28%) have required pulmonary artery conduit reconstruction for chronic right ventricular failure. Postrepair right ventricular/left ventricular pressure ratios were available in 19 patients, including five of seven who died and three of five who required reoperation. The ratios averaged 0.54, which indicates adequate relief of obstruction. The operative mortality rate from a number of series for transannular patch repair of tetralogy of Fallot averages 16%, ranging from 3% to 63%. However, when distal pulmonary artery problems are added as a risk factor this rate rises to 21%, and valved conduit insertion increases the mortality rate (Kirklin and Barratt-Boyes, 1986). Although not clearly superior, pulmonary artery conduit repair of tetralogy of Fallot achieves results comparable with those of transannular patch repair and is recommended as an alternative, particularly when distal pulmonary artery anatomy is abnormal or vascular resistance is elevated.

Blood Vessel Prosthesis↗
PubMed1995

Streptokinase and Heparin versus Heparin Alone in Massive Pulmonary Embolism: A Randomized Controlled Trial.

To test the efficacy of thrombolytic therapy in massive pulmonary embolism, we conducted a prospective randomized controlled trial. Eight patients were randomized to receive either 1,500,000 IU of streptokinase in 1 hour through a peripheral vein followed by heparin or heparin alone. All patients had major risk factors for deep vein thrombosis (DVT) and were considered to have high clinical suspicion for pulmonary embolism (PE). At baseline all patients had a similar degree of systemic arterial hypotension, pulmonary arterial hypertension, and right ventricular dysfunction. The time of onset of cardiogenic shock in both groups was comparable (2.25 +/- 0.5 hours in the streptokinase group and 1.75 +/- 0.96 hours in the heparin group). The four patients who were randomized to streptokinase improved in the first hour after treatment, survived, and in 2 years of follow-up are without pulmonary arterial hypertension. All four patients treated with heparin alone died from 1 to 3 hours after arrival at the emergency room (p = 0.02). Post-thrombolytic therapy the diagnosis of PE was sustained in the streptokinase group by high probability V/Q lung scans and proven DVT. A necropsy study performed in three patients in the heparin group showed massive pulmonary embolism and right ventricular myocardial infarction, without significant coronary arterial obstruction. The results indicate that thrombolytic therapy reduces the mortality rate of massive acute pulmonary embolism.

Journal Article↗
PubMed2006

The ventricular septum: the lion of right ventricular function, and its impact on right ventricular restoration.

OBJECTIVE: To evaluate the structure-function relationships of the right ventricle (RV) and septum and determine if the helical ventricular band model would define fiber orientation for maximal force response. Implications were made for right ventricular function. METHODS: The right ventricular free wall and biventricular septum were studied by inserting sonomicrometer crystals at different angulations to determine the maximum response of fiber shortening. These reactions were compared to the lateral left ventricular (LV) wall and further tested by use of positive and negative inotropic drug infusions. RESULTS: The maximum contraction of the free wall was achieved by placing crystals in the transverse orientation angulations, whereas oblique orientation allowed the maximal septal response. Fiber orientation angulation was the same for the LV free wall and septum. These angulations correlate with the MRI-related twisting actions of septal motion needed for ejection and suction for rapid filling. These findings have important impact, because they imply that the septum is 'lion of right ventricular function,' since septal twisting is essential when pulmonary vascular resistance is increased. The incidence of postoperative right heart failure due to septal dyssyncrony, with loss of septal twisting action from inadequate myocardial protection, is explored relative to RV free wall and septum function. Furthermore, early studies of right ventricular restoration in patients with RV dysplasia and RV failure after chronic pulmonary insufficiency following repair of Tetralogy of Fallot are described, with predominant attention directed toward rebuilding normal septal architecture and function. CONCLUSIONS: This experimental and clinical overview indicates that the septum is 'the lion of right ventricular function,' and implies that the use of this knowledge can become an important guideline for planning novel surgical geometric interventions after RV failure.

Heart Septum↗
PubMed1996

Usefulness of QRST isointegral maps for the diagnosis of right ventricular pressure overload in patients with surgically repaired tetralogy of Fallot complicated by right bundle block.

Right ventricular pressure overload was evaluated in 29 patients, 8-12 years old, with surgically repaired tetralogy of Fallot by using body surface QRST isointegral maps. In patients with right ventricular systolic pressure above 50 mmHg, the maxima of the isointegral maps tended to shift toward the lower right-hand region of the map. The maximum value was significantly correlated with right ventricular systolic pressure (r = .58; P < .01). There was a correlation between the right ventricular systolic pressure and the percentage +2SD and percentage +5SD departure areas, which are defined as the area (expressed as a percentage of the total chest area) in which the QRST integral values are greater than the normal mean +2SD or +5SD, respectively (r = .61 and .84, P < .01). The QRST isointegral map can be used to evaluate right ventricular pressure overload in postoperative patients with tetralogy of Fallot complicated by right bundle branch block. The percentage +5SD departure area is the most valuable parameter for the quantitative evaluation of the right ventricular systolic pressure.

Body Surface Potential Mapping↗
PubMed1998

Left ventricular regional systolic motion in patients with right ventricular pressure overload.

Left ventricular regional systolic motion was investigated in patients with right ventricular pressure overload and 10 controls using tagged cine magnetic resonance imaging. The regional shortening fraction was determined in four segments (septal, lateral, inferior, and anterior) on the short-axis image. An asynchrony index, nonhomogeneity of regional shortening, was calculated. Septal shortening in these patients was depressed, and showed an inverse correlation with the right-to-left ventricular peak pressure ratio (r=-0.80, P<0.01). Lateral shortening was greater in the patients than in the controls (P<0.01). The asynchrony index was significantly greater in the patients than in the controls (P<0.01), and correlated with the right-to-left systolic pressure ratio (r=0.64, P=0.02) and the left ventricular end-diastolic pressure (r=0.79, P<0.01). The altered distribution of regional circumferential shortening results in an increased heterogeneity of regional systolic motion. These findings may have important implications for the assessment of ventricular function in patients with right ventricular pressure overload.

Adult↗
PubMed2005

Right ventricular noncompaction in a neonate with complex congenital heart disease.

Ventricular noncompaction is a rare unclassified cardiomyopathy occurring because of arrest of the normal intrauterine compaction of the loose luminal component of the ventricular myocardium. There is limited data regarding its diagnosis and outcome in children. It is recognised, however, that right ventricular involvement is extremely rare. We report a case in which only the right ventricular myocardium was noncompacted, a situation which led to heart failure soon after birth.

Echocardiography↗
PubMed2001

Evaluation of right ventricular performance with a right ventricular ejection fraction thermodilution catheter and MRI in patients with pulmonary hypertension.

STUDY OBJECTIVES: We sought to compare catheter studies using a right ventricular ejection fraction (REF) catheter together with echocardiography and MRI in patients with pulmonary hypertension. PATIENTS AND METHODS: We compared hemodynamic findings, echocardiography, and MRI studies in 16 patients with pulmonary hypertension. Six healthy volunteers served as control subjects for the MRI studies. RESULTS: MRI imaging provided accurate assessment of cardiac output in all but two patients. As compared with MRI, the REF catheter constantly underestimated the REF and overestimated right ventricular volumes in patients with pulmonary hypertension. REF, end-systolic and end-diastolic right ventricular volumes, and right ventricular muscle mass, as determined by MRI, were almost identical in patients with preserved cardiac function and those with low-output failure. The only factor that was different in both groups was the severity of tricuspid regurgitation. CONCLUSION: Right ventricular dimensions and muscle mass do not differ in patients with pulmonary hypertension who have low cardiac output and those who do not. According to our results, the major determinant of cardiac output in these patients appears to be the severity of tricuspid regurgitation. The REF catheter provides invalid data on right ventricular dimensions in patients with pulmonary hypertension.

Adult↗
PubMed2002

Reversibility of electrophysiological changes induced by chronic high-altitude hypoxia in adult rat heart.

Recent studies indicate that regression of left ventricular hypertrophy normalizes membrane ionic current abnormalities. This work was designed to determine whether regression of right ventricular hypertrophy induced by permanent high-altitude exposure (4,500 m, 20 days) in adult rats also normalizes changes of ventricular myocyte electrophysiology. According to the current data, prolonged action potential, decreased transient outward current density, and increased inward sodium/calcium exchange current density normalized 20 days after the end of altitude exposure, whereas right ventricular hypertrophy evidenced by both the right ventricular weight-to-heart weight ratio and the right ventricular free wall thickness measurement normalized 40 days after the end of altitude exposure. This morphological normalization occurred at both the level of muscular tissue, as shown by the decrease toward control values of some myocyte parameters (perimeter, capacitance, and width), and the level of the interstitial collagenous connective tissue. In the chronic high-altitude hypoxia model, the regression of right ventricular hypertrophy would not be a prerequisite for normalization of ventricular electrophysiological abnormalities.

Aging↗
PubMed1999

Antecedent angina pectoris as a predictor of better functional and clinical outcomes in patients with an inferior wall acute myocardial infarction.

We examined whether angina pectoris (AP) occurring shortly before the onset of acute myocardial infarction (AMI) can render the right ventricle and the conducting tissue resistant to ischemia in 75 patients with an inferior wall AMI. Each patient had total occlusion in the proximal right coronary artery and underwent successful coronary angioplasty < or =24 hours from the onset. We divided patients into 2 groups based on presence or absence of antecedent AP < or =24 hours before the system onset: group 1 (absent) = 57 patients; group 2 (present) = 18 patients. Collateral circulation was more frequently observed in group 2 than in group 1 (group 1 vs 2, 28% vs 61%, p <0.01). Elevation in ST segment > or =1 mm in lead V4R, hemodynamic right ventricular dysfunction, and frequency of high-degree heart block were more frequent in group 1 than in group 2 (75% vs 44%, 79% vs 39%, 53% vs 11%, p <0.05, respectively). Multivariate analysis demonstrated that antecedent AP is the only factor related to these complications. Thus, episodes of AP occurring shortly before onset may restrain development of ischemic damage of the right ventricle and conducting tissue, and are associated with better clinical and functional outcomes among patients with an inferior wall AMI.

Acute Disease↗
PubMed1997

Right ventricular function before and after percutaneous balloon mitral valvuloplasty.

Aim of this study was to evaluate right ventricular performance in patients with mitral stenosis and its modification by balloon valvuloplasty. Right ventricular volumes of 24 patients with postrheumatic mitral stenosis were determined by thermodilution 1 or 2 days before and 1 or 2 days after valvuloplasty. Right ventricular ejection fraction at rest was 43 (36-47)% (median and interquartile range). Right ventricular end-diastolic volume was 100 (86-119) ml/m2. Supine bicycle exercise (50 Watt) reduced right ventricular ejection fraction to 30 (29-37)% (P < 0.0001) and increased right ventricular end-diastolic volume to 124 (112-141) ml/m2 (P < 0.0001). At rest, right ventricular ejection fraction correlated inversely with pulmonary vascular resistance (r = -0.64, P < 0.0001), while no significant correlation with mitral valve area was found. Valvuloplasty increased right ventricular ejection fraction at rest to 48 (44-50)% (P < 0.005), and during exercise to 42 (38-45)% (P < 0.0001). This improvement of right ventricular ejection fraction correlated inversely with the value of this parameter before valvuloplasty (r = -0.88, P < 0.0001) and with the gain in stroke volume (r = 0.57, P < 0.01). The right ventricular function curve, disturbed before commissurotomy, was reestablished by the procedure. In conclusion, at the here investigated stage of mitral stenosis right ventricular function is reversibly impaired. This is predominantly caused by the hemodynamic consequences of the valvular defect and not by an impairment of right ventricular myocardial function.

Adult↗
PubMed2005

Congenital right atrial diverticulum, atrial septal defect within the oval fossa, and complex pulmonary valvar obstruction in an infant with chromosome 8(p23.1) deletion.

We describe an unusual right atrial aneurysm, occurring with progressive obstruction of the right ventricular outflow tract and increasing cyanosis, in a nine-month-old boy with chromosome 8(p23.1) deletion. Surgical resection of the diverticulum, and relief of the right ventricular obstruction, was successful, although impairment of right ventricular compliance persists after 19 months follow-up.

Chromosome Deletion↗