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PubMed2006

Impact of atrial septal defect closure on right ventricular performance.

BACKGROUND: Atrial septal defect (ASD) closure is one of the most representative cardiac operations, but there have been few assessments of right ventricular (RV) performance during the perioperative period. METHODS AND RESULTS: Using transesophageal echocardiography with automated border detection system, the RV pressure - area (P-A) loops were measured in 6 patients immediately before and after an ASD closure. Multiple RV P-A loops obtained by the inferior vena cava occlusion technique were used to evaluate end-systolic maximal elastance (Ees), preload recruitable stroke work (MEW), external work (EW) and the systolic P-A area (PAA). Ees and MEW are indices of contractility, and PAA represents the total mechanical energy. RV Ees and MEW did not change after the operation (from 3.36 +/- 2.20 to 3.70 +/- 2.04 mmHg/cm2, p = 0.4; and from 10.3 +/- 5.0 to 10.5 +/- 4.5 mmHg, p = 0.8, respectively), whereas EW and PAA were significantly reduced (from 121.4 +/- 77.4 to 48.7 +/- 26.6 mmHg/cm2, p < 0.05; and from 274.8 +/- 212.8 to 92.7 +/- 52.8 mmHg/cm2, p < 0.05, respectively). ASD closure reduced the EW and total mechanical energy of the right ventricle without influencing contractility. CONCLUSIONS: These results indicate that ASD closure preserved RV function and reduced RV myocardial oxygen consumption. Assessment of the RV P-A relationship in the operating room demonstrates the beneficial effects of ASD closure on RV performance.

Adult↗
PubMedDate not supplied

[One general pattern in the development of pathologic processes in the heart in various types of myocardial involvement].

Experiments on 50 rabbits were made to study left and right ventricular function and ultrastructure of the heart in its diffuse and focal disease, overload of the intact heart. In the middle stage of the above conditions there was a distinct dyscoordination of the heart function and the growth of differences in the heart ventricles ultrastructures. This phenomenon may underlie the rise of intracardiac and general hemodynamic disturbances.

Animals↗
PubMed1998

Evaluation of right ventricular systolic pressure during incremental exercise by Doppler echocardiography in adults with atrial septal defect.

STUDY OBJECTIVES: Pulmonary hypertension is the most important complication in patients with atrial septal defect (ASD), but its role in limiting exercise has not been examined. This study sought to evaluate exercise performance in adults with ASD and determine the contribution of elevated pulmonary artery pressure in limiting exercise capacity. DESIGN: We used Doppler echocardiography during exercise in 10 adults (aged 34 to 70 years) with isolated ASD (New York Heart Association class I, II) and an equal number of matched control subjects. Incremental exercise was performed on an electrically braked upright cycle ergometer. Expired gases and VE were measured breath-by-breath. Two-dimensional and Doppler echocardiographic images were obtained at rest prior to exercise to determine ASD size, stroke volume (SV), shunt ratio (Qp:Qs), right ventricular outflow tract (RVOT) size, and right ventricular systolic pressure at rest (RVSPr). Doppler echocardiography was repeated at peak exercise to measure right ventricular systolic pressure during exercise (RVSPex). RESULTS: Resting echocardiography revealed that RVOT was larger (21+/-4 vs 35+/-8 mm, mean+/-SD; p=0.0009) and RVSPr tended to be higher (17+/-8 vs 31+/-8 mm Hg; p=0.08) in ASD; however, left ventricular SV was not different (64+/-23 vs 58+/-23 mL; p>0.05), compared with control subjects. Despite normal resting left ventricular function, ASD patients had a significant reduction in maximum oxygen uptake (VO2max) (22.9+/-5.4 vs 17.3+/-4.2 mL/kg/min; p=0.005). RVSPex was higher (19+/-8 vs 51+/-10 mm Hg; p=0.001) and the mean RVSP-VO2 slope (1+/-2 vs 18+/-3 mm Hg/L/min; p=0.003) and intercept (17+/-4 vs 27+/-4 mm Hg; p=0.05) were higher in the ASD group. VO2max correlated inversely with both RVSPr (r=-0.69; p=0.007) and RVSPex (r=-0.67; p=0.01). CONCLUSION: These findings suggest that adults with ASD have reduced exercise performance, which may be associated with an abnormal increase in pulmonary artery pressure during exercise.

Adult↗
PubMedDate not supplied

Mechanical circulatory support for perioperative donor heart failure.

Perioperative acute donor heart failure can be caused by various factors, such as recipient pulmonary hypertension, marginal donor heart function, or immunologic mismatch. Of 265 patients who underwent orthotopic transplantation, four received mechanical support for acute perioperative donor heart failure. In two patients with reactive pulmonary hypertension, right heart bypass (RHB) with a centrifugal pump was used for 53 and 36 hr, respectively. One patient who experienced biventricular donor heart failure was supported for three days with an intraarterial, transvalvular, axial-flow left ventricular assist device (LVAD). Circulation was supported effectively with the LVAD, despite an initial absence of right ventricular function. The fourth patient, who had signs of heart failure, received intraaortic balloon pump support for 24 hr after transplantation. All four patients were weaned from circulatory support, and heart function was restored in each; the mean left ventricular ejection fraction was 63% (range from 57 to 71%). One patient died of fungal infection 14 days after being weaned from pump support, another died of lymphoma two months after support was discontinued, and the remaining two patients are well 9 and 18 months after transplantation. In cases of acute donor heart dysfunction, temporary mechanical assistance is a reliable option for supporting the circulation during heart recovery.

Adult↗
PubMed1998

Effects of amrinone, a phosphodiesterase inhibitor, on right ventricular/arterial coupling immediately after cardiac operations.

OBJECTIVE: Amrinone, a selective phosphodiesterase III inhibitor, is reported to have a potent inotropic effect on the left ventricle, but the effects of this drug on right ventricular contractility in the clinical setting are unknown. The concept of ventricular/arterial coupling was applied to investigate the effects of amrinone on right ventricular contractility and afterload with transesophageal echocardiography. METHODS AND RESULTS: The study was performed in the intensive care unit with 11 patients who had undergone cardiac operations. Right ventricular cross-sectional area was measured with transesophageal echocardiography equipped with a capability of automated border detection as a surrogate for right ventricular volume. Multiple pressure-area loops were obtained by reducing preload to measure end-systolic elastance and effective arterial elastance. External work and pressure-volume area were also measured to calculate the efficiency of energy transfer from pressure-volume area to external work. Nitroprusside (0.3 to 0.5 microg x kg(-1) x min(-1)) and amrinone (1 mg x kg(-1) intravenously followed by 10 microg x kg(-1) x min(-1)) were administered. With nitroprusside infusion, end-systolic elastance and effective arterial elastance remained unchanged (end-systolic elastance, 4.73 +/- 2.18 mm Hg/cm2 to 4.65 +/- 2.09 mm Hg/cm2; effective arterial elastance, 8.05 +/- 3.84 mm Hg/cm2 to 7.70 +/- 3.64 mm Hg/cm2). In contrast, amrinone reduced afterload (effective arterial elastance, 8.82 +/- 3.99 mm Hg/cm2 to 7.05 +/- 4.01 mm Hg/cm2, p = 0.004) and enhanced contractility (end-systolic elastance, 4.47 +/- 1.79 mm Hg/cm2 to 6.56 +/- 2.22 mm Hg/cm2, p = 0.007). Consequently, amrinone decreased the ventricular/arterial coupling ratio (effective arterial elastance/end-systolic elastance, 2.40 +/- 1.45 to 1.16 +/- 0.63, p = 0.009) and improved the efficiency of energy transfer (external work/pressure-volume area, 0.44 +/- 0.15 to 0.54 +/- 0.15, p = 0.013). CONCLUSIONS: Right ventricular pressure-area relations obtained with transesophageal echocardiography could successfully separate the simultaneous change in right ventricular systolic mechanics and afterload caused by amrinone. Amrinone caused enhancement of right ventricular contractility and afterload reduction.

Adult↗
PubMed1999

Noninvasive estimation of pulmonary artery diastolic pressure in patients with tricuspid regurgitation by Doppler echocardiography.

OBJECTIVES: The purpose of this study was to determine whether Doppler echocardiographic assessment of right ventricular pressure at the time of pulmonary valve opening could predict pulmonary artery diastolic pressure. BACKGROUND: Doppler echocardiography has been used to estimate right ventricular systolic pressure noninvasively. Because right ventricular and pulmonary artery diastolic pressure are equal at the time of pulmonary valve opening, Doppler echocardiographic estimation of right ventricular pressure at this point might provide an estimate of pulmonary artery diastolic pressure. METHODS: We studied 31 patients who underwent right heart catheterization and had tricuspid regurgitation. Pulmonary flow velocity was recorded by pulsed wave Doppler echocardiography, and tricuspid regurgitant velocity was recorded by continuous wave Doppler echocardiography. The time of pulmonary valve opening was determined as the onset of systolic flow in the pulmonary artery. Tricuspid velocity at the time of pulmonary valve opening was measured by superimposing the interval between the onset of the QRS complex on the ECG and the onset of pulmonary flow on the tricuspid regurgitant envelope. The tricuspid gradient at this instant was calculated from the measured tricuspid velocity using the Bernoulli equation. This gradient was compared to the pulmonary artery diastolic pressure obtained by right heart catheterization. MEASUREMENTS AND RESULTS: The pressure gradient between the right atrium and right ventricle obtained at the time of pulmonary valve opening ranged from 9 to 31 mm Hg (mean, 19+/-5) and correlated closely with invasively measured pulmonary artery diastolic pressure (range, 9 to 36 mm Hg; mean, 21+/-7 mm Hg; r = 0.92; SEE, 1.9 mm Hg). CONCLUSION: Doppler echocardiographic measurement of right ventricular pressure at the time of pulmonary valve opening is a reliable noninvasive method for estimating pulmonary diastolic pressure.

Aged↗
PubMed2006

Echocardiographically estimated left ventricular end-diastolic and right ventricular systolic pressure in normotensive healthy individuals.

AIM: To study the effect of aging on and the relationship between echocardiographically estimated left ventricular (LV) filling pressure and estimated right ventricular (RV) systolic pressure among healthy normotensive individuals. METHODS: We analyzed 249 healthy individuals (aged 18-82 years, 52% men) with normal echocardiographic findings and reliably measurable tricuspid regurgitation gradients. Subjects with blood pressure >140/90 mmHg and/or LV hypertrophy were excluded. LV & RV dimensions and LV mass were measured with M-mode echocardiography. Atrial (A) volumes were determined with the area-length method. Diastolic function was assessed with transmitral Doppler and mitral annulus tissue Doppler. The ratio of transmitral early peak velocity to early diastolic mitral annulus velocity (E/E') was used as estimation of LV filling pressure. The transtricuspid Doppler gradient was used to estimate RV end-systolic pressure. RESULTS: Even in normotensive individuals aging was accompanied by an increase in LV mass and LA dimensions and an increase in relaxation abnormalities. E/E' increased with every decade: from 7.8 for age 18-35 years to 10.9 for age > or =75 years (p<0.0001) as did the transtricuspid gradient: from 18.3 mmHg for age 18-35 years to 25.8 mmHg for age > or =75 years (p<0.0001). Linear regression showed that estimated RV systolic pressure was independently predicted by age, LA volume, LV systolic function and E/E'. CONCLUSION: Among normotensive healthy individuals both E/E' and tricuspid regurgitation gradients increase significantly with aging. Moreover the E/E' ratio was independently predicting the tricuspid regurgitation gradient. These findings support the need for further studies defining age specific normal values.

Adolescent↗
PubMed1996

Measurement of pulmonary artery diastolic pressure from a right ventricular pressure transducer in patients with heart failure.

Recent studies have demonstrated that pulmonary artery diastolic (PAD) pressure can be measured from a transducer positioned in the right ventricle (RV) based on the finding that PAD and RV pressures are equal at the time of pulmonary valve opening, which is associated with the time of maximum positive rate of pressure development (dP/dtmax) in the ventricle. The objective of this study was to assess the correlation between estimated PAD (ePAD) pressure, obtained through a RV transducer, and actual PAD (aPAD) pressure in patients with heart failure who have abnormal hemodynamics, reduced systolic function, and variable degrees of mitral regurgitation (MR) and tricuspid regurgitation (TR). Simultaneous measurements of pulmonary artery and RV pressures were obtained with a high-fidelity Millar catheter (Millar Instruments, Houston, TX) in 10 patients with New York Heart Association class III-IV heart failure who were being evaluated for cardiac transplantation. The overall correlation between ePAD and aPAD pressures was .92 (R2 = .878). This was not significantly different during the Valsalva maneuver (r = .96, R2 = .943), submaximal bicycle exercise (r = .87, R2 = .756), or infusions of dobutamine and nitroglycerin (r = .82, R2 = .730). The overall average difference between the average ePAD (24.6 +/- 7.0 mmHg) and aPAD (23.6 +/- 7.0 mmHg) pressures was 1.0 +/- 3.4 mmHg. The average difference between the two pressures in patients with mild to severe MR or TR was not different compared to those patients with no or trace MR or TR. The estimation of PAD pressure from an RV transducer is valid in patients with heart failure who have abnormal hemodynamics, reduced systolic function, and variable degrees of MR and TR. This correlation was observed at rest and during several provocative maneuvers. These data will be important for the development of a chronic, implantable hemodynamic monitor for patients with heart failure.

Adult↗
PubMed2003

Postinfarction remodeling of the heart: types of pathomorphological changes in the right ventricle.

New geometric characteristics of the right ventricle depended on the localization of macrofocal transmural scars in the left ventricle of postinfarction heart. Most pronounced changes in the right ventricle were observed during dilatational and hypertrophic remodeling of the heart. The increase and decrease in the volume were most frequently occurring and pathognomonic forms of pathomorphological changes in the right ventricle. Dilatational remodeling was accompanied by a decrease in the volume of the right ventricle. The increase in the volume of this ventricle was typical of hypertrophic remodeling. Pathological variability in the right ventricle underlies the development of severe disturbances in intracardiac hemodynamics, i.e., patho- and thanatogenesis of postinfarction heart.

Aged↗
PubMed1998

Determination of right ventricular structure and function in normoxic and hypoxic mice: a transesophageal echocardiographic study.

BACKGROUND: Noninvasive cardiac evaluation is of great importance in transgenic mice. Transthoracic echocardiography can visualize the left ventricle well but has not been as successful for the right ventricle (RV). We developed a method of transesophageal echocardiography (TEE) to evaluate murine RV size and function. METHODS AND RESULTS: Normoxic and chronically hypoxic mice (F(IO2)=0.11, 3 weeks) and agarose RV casts were scanned with a rotating 3.5F/30-MHz intravascular ultrasound probe. In vivo, the probe was inserted in the mouse esophagus and withdrawn to obtain contiguous horizontal planes at 1-mm intervals. In vitro, the probe was withdrawn along the left ventricular posterior wall of excised hearts. The borders of the RV were traced on each plane, allowing calculation of diastolic and systolic volumes, RV mass, RV ejection fraction, stroke volume, and cardiac output. RV wall thickness was measured. Echo volumes obtained in vitro were compared with cast volumes. Echo-derived cardiac output was compared with measurements of an ascending aortic Doppler flow probe. Echo-derived RV free wall mass was compared with true RV free wall weight. There was excellent agreement between cast and TEE volumes (y=0.82x+6.03, r=0.88, P<0.01) and flow-probe and echo cardiac output (y=1.00x+0.45, r=0.99, P<0.0001). Although echo-derived RV mass and wall thickness were well correlated with true RV weight, echo-derived RV mass underestimated true weight (y=0.53x+2.29, r=0.81, P<0.0001). RV mass and wall thickness were greater in hypoxic mice than in normoxic mice (0.78+/-0.19 versus 0.51+/-0.14 mg/g, P<0.03, 0.50+/-0.03 versus 0.38+/-0.03 mm, P<0.04). CONCLUSIONS: TEE with an intravascular ultrasound catheter is a simple, accurate, and reproducible method to study RV size and function in mice.

Animals↗
PubMed1990

Long-term results of total repair of tetralogy of Fallot in childhood.

Between 1958 and 1977, 170 children aged 10 years or less underwent total repair of tetralogy of Fallot at The Johns Hopkins Hospital. Follow-up data were obtained on 128 (90%) of the 143 who survived the operation at 10 to 28 years postoperatively (mean follow-up, 18 years). All patients completed an extensive questionnaire, and 59 returned for a thorough evaluation consisting of a history and physical examination, electrocardiogram, 24-hour Holter monitoring, exercise stress testing, pulmonary function testing, and two-dimensional and Doppler echocardiography. Late survival was excellent with only two of four known late deaths due to cardiac-related causes and with all 59 patients in New York Heart Association class I or II. None had cyanosis or clubbing. Normal sinus rhythm was present in 90%. One patient had complete heart block, and 75% had right bundle-branch block on the electrocardiogram. Right ventricular function was normal by echocardiography in 78%. Residual mild to moderate pulmonary stenosis was noted by Doppler study in 8 patients. Pulmonary regurgitation was present in 78%, but in only 11 patients was it graded as moderate and in none was it severe. Stress testing documented the excellent functional status of most patients, with 92% of predicted exercise time and 94% of maximum heart rate being attained. In the few who had impaired cardiac performance, this correlated best with moderate pulmonary regurgitation. Although the overall late functional status of patients undergoing repair in the first decade of life was very good, these patients need continued follow-up to assess the severity of pulmonary regurgitation and the need of possible intervention.

Adult↗
PubMed1992

Importance of left ventricular function and systolic ventricular interaction to right ventricular performance during acute right heart ischemia.

To determine whether modulation of systolic ventricular interaction influences right ventricular performance during right heart ischemia, the effects of septal ischemia and inotropic stimulation were studied in 15 dogs in an open chest preparation. Right coronary branch occlusions led to right ventricular dilation and free wall dyskinesia, reversed septal curvature and reduced left ventricular diastolic volume. In systole, the septum thickened but bulged paradoxically into the right ventricle generating an active but depressed right ventricular systolic pressure (28.9 +/- 5.5 to 22.1 +/- 4.5 mm Hg), with associated decreases in right ventricular stroke work (5.66 +/- 0.94 to 1.92 +/- 0.53 g.m/m2) and left ventricular systolic pressure (123 +/- 11 to 80 +/- 10 mm Hg). Septal ischemia induced systolic septal thinning, left ventricular dilation and decreased left ventricular systolic pressure (80 +/- 10 to 55 +/- 10 mm Hg) and stroke work. Although the extent of paradoxic septal displacement increased, there were further decrements in right ventricular systolic pressure (22.1 +/- 4.5 to 18.7 +/- 4.3 mm Hg) and stroke work (1.92 +/- 0.53 to 0.7 +/- 0.2 g.m/m2). Dopamine infusion augmented left ventricular free wall contraction and increased left ventricular systolic pressure (55 +/- 10 to 172 +/- 17 mm Hg) and stroke work. Although systolic septal thinning persisted, the extent of paradoxic septal displacement increased strikingly and, despite continued right ventricular free wall dyskinesia, right ventricular systolic pressure increased (18.7 +/- 4.3 to 39.6 +/- 6.2 mm Hg) as did right ventricular stroke work (0.7 +/- 0.2 to 7 +/- 1.6 g.m/m2).(ABSTRACT TRUNCATED AT 250 WORDS)

Acute Disease↗
PubMed2002

Hemodynamic changes during displacement of the beating heart using epicardial stabilization for off-pump coronary artery bypass graft surgery.

OBJECTIVE: To evaluate the hemodynamic alterations during off-pump coronary artery bypass graft surgery to determine the degree of impairment caused and the techniques to rectify them. DESIGN: Prospective, observational cohort study performed from January 2000 through September 2000. PARTICIPANTS: Patients (n = 500) with coronary artery disease undergoing multivessel off-pump coronary artery bypass graft surgery using the Octopus tissue stabilizer (Medtronic, Inc, Minneapolis, MN). Unstable patients with ongoing ischemia were excluded from the study. INTERVENTIONS: All patients were monitored with radial artery and pulmonary artery catheters and continuous transesophageal echocardiography monitoring with a multiplane transducer. The perioperative requirement of an intracoronary shunt, inotropes, or an intra-aortic balloon pump was noted. The effect of the Trendelenburg position and fluids on hemodynamics was observed. The need for defibrillation and institution of emergency cardiopulmonary bypass were major endpoints to determine the inability of the patient to tolerate displacement of the heart. MEASUREMENTS AND MAIN RESULTS: Mean patient age was 59.3 +/- 11.6 years. There were 204 (40%) patients in the high-risk category; 54 (10.8%) patients had left ventricular ejection fraction <25%. The mean number of grafts was 2.7 +/- 0.8. Vertical displacement of the heart to access the lateral and inferior walls decreased the mean arterial pressure by 18 +/- 4% (p < 0.01), with a concomitant increase in central venous pressure of 66 +/- 18% (p < 0.001). The stroke volume and the cardiac index were reduced by 35.7 +/- 11% (p < 0.001) and 45 +/- 13% (p < 0.001). On transesophageal echocardiography, there was development of new regional wall motion abnormalities in 59.2% and a decrease in global left ventricular functions in 61.2%. The use of inotropes was highest during anastomosis on the posterior wall-78.4% compared with 21.9% for the anterior wall. An intra-aortic balloon pump was used in 55 (11.2%) patients, and 7 (0.71 %) patients had to be put on emergency CPB. The in-hospital mortality was 1.2%. CONCLUSION: Most patients had hemodynamic changes easily correctable by fluids and inotropes. Monitoring of left ventricular and right ventricular function by transesophageal echocardiography enhances safety of the procedure and is recommended. The use of the Octopus II tissue stabilizer proved to be a safe and versatile means to stabilize the heart during off-pump coronary artery bypass procedures, especially in high-risk patients.

Cardiopulmonary Bypass↗
PubMedDate not supplied

Right ventricular dysfunction secondary to acute massive pulmonary embolism detected by helical computed tomography pulmonary angiography.

OBJECTIVE: Acute massive pulmonary embolism causes abrupt pulmonary arterial hypertension and right ventricular dysfunction (RVD). Patients with RVD have a worse prognosis than those with normal right ventricular function. Consequently, recognizing the RVD at the time of pulmonary embolism is useful for risk stratification and enables more aggressive therapy. The study compared the accuracy of helical computed tomographic (CT) scans with echocardiography in the detecting of RVD in patients with acute massive pulmonary embolism. MATERIALS AND METHODS: Specifically, this work reviewed the CT pulmonary angiograms of 14 patients who were positive for acute massive pulmonary embolism during a 52-month period. CT scans were reviewed for findings indicating RVD. Scans were considered positive for RVD if the right ventricle was dilated or there was leftward shift of the interventricular septum. Echocardiographic reports serving as the reference standard for the diagnosis of RVD were also reviewed. CT study results were then correlated with echocardiography results. RESULTS: Among 14 patients with massive pulmonary embolism, echocardiography identified 12 patients having RVD, whereas the remaining two patients were negative for RVD. Meanwhile, CT correctly identified 11 of 12 patients as having RVD, and was negative for RVD in the remaining 3 patients. Correlated with echocardiography, CT scan for RVD detection had a sensitivity of 91.6% and a specificity of 100%. CONCLUSIONS: CT can accurately detect RVD in patients with acute massive pulmonary embolism. However, this result requires confirmation using a larger prospective cohort study.

Adult↗
PubMed2006

Percutaneous pulmonary valve implantation.

Percutaneous implantation of valves is a fast evolving field in interventional cardiology. Acquired pulmonary valve disease is uncommon; however, right ventricular outflow tract dysfunction is one of the common indications of reoperations during late follow-up of repaired congenital heart disease. The impact of pulmonary valvular dysfunction on right ventricular function during late follow-up of patients with adult congenital heart disease has led to earlier intervention. The importance of a technique with lower morbidity and mortality, good patient acceptance, and efficacy that is comparable with surgery cannot be underestimated. Percutaneous pulmonary valve implantation uses a valved-stent assembly implanted by means of a double balloon catheter delivery system. The device is a bovine jugular venous valve sutured inside a platinum-iridium. In a series of 58 consecutive patients, we implanted this valve successfully with good clinical and hemodynamic results and no mortality on long-term follow-up. Early device designs led to reintervention by surgical explantation in the early experience, and a second percutaneous pulmonary valve implantation during latter experience. This exciting new technique will lead to review of the current indications and timing of intervention for right ventricular outflow tract dysfunction in late follow-up of congenital heart disease.

Adolescent↗
PubMed2006

[Anatomic correction of the congenitally corrected transposition of the great vessels by the first successful "double switch" operation in Hungary].

Congenitally corrected transposition of the great arteries is a rare defect characterized by discordant atrioventricular and ventriculoarterial connections. Symptoms result from one or a combination of associated cardiovascular malformations, including ventricular septal defect, pulmonary stenosis or atresia, tricuspid valve dysfunction, dextrocardia, hypoplastic left or right ventricle. Correcting exclusively the associated defects, leaving the morphologic right ventricle in systemic position, will determine the patient's life-long prognosis. Anatomic repair by double switch technique may improve survival of patients with congenitally corrected transposition of the great arteries by establishing the morphologic left ventricle in the systemic circulation. A 3-year-old girl with congenitally corrected transposition of the great arteries, ventricular septal defect, hypoplastic right ventricle, and previous palliative procedure was corrected by double switch technique and patch closure of ventricular septal defect. The authors prefer the double switch procedure inspite of its many surgical challenges because it has very good long-term outcome. The more simple surgery namely the operation of only associated cardiac defects will involve the possibility of deterioration of right ventricular function.

Cardiac Surgical Procedures↗
PubMed1995

Risk factors of cardiac arrest in arrhythmogenic right ventricular dysplasia.

Arrhythmogenic right ventricular dysplasia is an important cause of ventricular arrhythmia with a potential risk of sudden cardiac death in a young population. In order to define risk factors of cardiac arrest, angiographic and electrophysiological data from 60 patients with angiographically documented arrhythmogenic right ventricular dysplasia (of whom 20 also had spontaneous non-sustained ventricular arrhythmias, 27 sustained ventricular tachycardia and 13 suffered cardiac arrest with documented ventricular fibrillation before resuscitation) were analysed. There were no statistically significant differences in right ventricular volume, global right ventricular ejection fraction (RVEF), right ventricular structure, mean age at the time of diagnosis and angiographic left ventricular contraction abnormalities in the subgroups of patients with different forms of spontaneous arrhythmias. Only in a subgroup of patients with cardiac arrest and inducible sustained ventricular tachycardia did right ventricular volume (P < 0.05), ejection fraction (P < 0.001) and the amount of structural changes (P < 0.01) reveal significant results. A subgroup of patients with structural alterations and a low level of right ventricular function is at a high risk of cardiac arrest, although strenuous exercise and sport remain most important risk factors.

Adolescent↗