Assessment of longitudinal systolic ventricular dysfunction and asynchrony using velocity vector imaging in children with a single right ventricle.

Assessment of ventricular dysfunction and asynchrony is very important in predicting the outcome for children with a single right ventricle. However, the assessment is inaccurate and subjective because of the unusual ventricular shape. This study aimed to evaluate the feasibility and clinical value of velocity vector imaging for assessing longitudinal systolic ventricular dysfunction and intraventricular asynchrony in children with a single right ventricle. The study enrolled 36 children with a single right ventricle and 36 age-matched children with a normal heart. Peak systolic velocity, peak displacement, strain, strain rate, time to peak systolic velocity, and time to peak systolic strain were measured via velocity vector imaging using the Siemens Sequoia C512 echocardiography instrument. The maximum positive rate of ventricular pressure change (Max [dp/dt]) was obtained by cardiac catheterization for all the children with a single right ventricle. In the children with a single right ventricle, the maximal temporal differences and the standard deviations of the times to peak systolic velocity and peak systolic strain were higher (P < 0.01) than in the children with a normal heart. Moreover, the strain and strain rate values were significantly lower in all six segments (P < 0.05). The strain rate of the basal segment adjacent to the rudimentary chamber correlated best with Max (dp/dt) (r = 0.86; P < 0.01). Longitudinal systolic dysfunction and intraventricular asynchrony could be assessed accurately using velocity vector imaging in children with a single right ventricle.

Ventricular response to stress predicts outcome in adult patients with a systemic right ventricle.

BACKGROUND: Previous studies demonstrated that ventricular response to stress cardiovascular magnetic resonance (CMR) is frequently abnormal in patients with a systemic right ventricle (RV). However, the clinical implications of these findings remained unknown. We sought to evaluate whether abnormal response to stress CMR predicts adverse outcome in patients with a systemic RV. METHODS: Thirty-nine adult patients (54% male; mean age 26, range 18-65 years) with a systemic RV underwent stress CMR to determine the response of RV volumes and ejection fraction (EF). During follow-up, cardiac events, defined as hospitalization for heart failure, cardiac surgery, aborted cardiac arrest, or death, were recorded. The prognostic value of an abnormal response to stress, defined as lack of a decrease in RV end-systolic volume (ESV) or lack of an increase in RV EF, was assessed. RESULTS: We frequently observed an abnormal response to stress, as RV ESV did not decrease in 17 patients (44%), and RV EF did not increase in 15 patients (38%). After a mean follow-up period of 8.1 years, 8 (21%) patients had reached the composite end point. The inability to decrease RV ESV during stress was predictive for cardiac events with a hazard ratio of 2.3 (95% CI 1.19-88.72, P = .034), as was the inability to increase RV EF with a hazard ratio of 2.3 (95% CI 1.31-81.59, P = .027). CONCLUSIONS: Stress CMR potentially has important prognostic value in patients with a systemic RV. Patients with a systemic RV who show abnormal cardiac response to stress have a substantially higher risk of adverse outcome.

Rationale and design of a trial on the effect of angiotensin II receptor blockers on the function of the systemic right ventricle.

BACKGROUND: Angiotensin II receptor blockers have been proven to be beneficial in left ventricular failure. In patients with a morphologic right ventricle supporting the systemic circulation, its efficacy has not yet been established. METHODS: We designed a multicenter, prospective, randomized, double-blind, placebo-controlled trial studying the effect of valsartan in patients with a systemic right ventricle due to a congenitally or surgically corrected transposition of the great arteries. The primary end point is the change in right ventricular ejection fraction as measured by cardiovascular magnetic resonance or multidetector row cardiac computed tomography in case of pacemaker patients. CONCLUSION: This large prospective, double-blind, randomized, placebo-controlled trial will establish the role of angiotensin II receptor blockers (valsartan) in the treatment of patients with a systemic right ventricle.

Off-pump coronary artery bypass in a patient with congenital absence of the left pericardium and an extremely left-rotated heart.

Congenital anomalies of the pericardium are extremely rare and often difficult to diagnose. We describe a 66-year-old woman presenting with multivessel coronary artery disease and right heart insufficiency complicated by an extremely left-rotated heart due to a congenital absence of left pericardium. A multidetector row computerized tomographic study was performed to evaluate the torsion of the heart as well as the physical relationship between the left internal thoracic artery and the left coronary system. Off-pump coronary surgery was scheduled. Both the operative and postoperative courses were uneventful and the patient was discharged after 11 days.

B-type natriuretic peptide in patients with systemic right ventricle.

OBJECTIVE: To examine the diagnostic value of B-type natriuretic peptide (BNP) in patients with systemic right ventricle. METHODS: Plasma BNP levels were measured in 48 outpatients with D-transposition of the great arteries after atrial switch procedure or congenitally corrected transposition of the great arteries. RESULTS: Plasma BNP was < or =20 pg/ml in 52% and <200 pg/ml in all patients, significantly higher in females compared to males (p = 0.004), and positively correlated with age (r = 0.3, p = 0.04). New York Heart Association class I patients had significantly lower BNP than class II patients (p = 0.03). A positive correlation was found between BNP and severity of tricuspid regurgitation (r = 0.5, p < 0.001). Plasma BNP was weakly, negatively correlated to maximum exercise capability, peak oxygen uptake, maximum heart rate during exercise testing and minimal heart rate in the 24-hour Holter monitoring. Additionally, BNP was negatively correlated with flow velocity across the pulmonary valve in patients with congenitally corrected transposition of the great arteries (r = -0.81, p = 0.03). CONCLUSION: Plasma BNP is normal in patients with systemic right ventricle, but increases with deterioration of clinical status and decreasing exercise capability. BNP is positively correlated with severity of tricuspid regurgitation, a prognostic factor for survival.

Borderline left ventricle.

The first problem to solve when dealing with the topic 'borderline left ventricle' is to find the appropriate definition. Several parameters have been taken into consideration, either morphometric (diameter of the mitral valve, indexed mitral valve area, left ventricular inflow dimension, left ventricular cross-sectional area, ratio between the apex-to-base left ventricular dimension and right ventricular dimension, left ventricular long axis to heart long axis ratio, left ventricular end diastolic volume, left ventricular mass index, ratio of the right/left ventricular wall thickness, presence of endocardial fibroelastosis, cardiac apex not formed by the left ventricle, diameter of the ventriculo-aortic junction, diameter of the aortic valve annulus and indexed aortic root diameter) as well as functional (left ventricular ejection fraction, left ventricular end diastolic pressure, mean pulmonary artery pressure, direction of the blood flow in the ascending aorta and at the level of the patent ductus arteriosus). Pre-operative determination whether the left ventricle is adequate to sustain the systemic circulation, or it may became adequate with the available surgical approaches, and therefore a bi-ventricular type of repair is feasible, can be extremely difficult, particularly in the presence of a 'borderline left ventricle'. In the clinical practice pediatric cardiologists and cardiac surgeons are faced with the problem of the 'borderline left ventricle' in four different groups of congenital heart defects: (a) aortic valve stenosis, (b) aortic coarctation, with or without hypoplastic aortic arch, (c) hypoplastic left heart complex, (d) right ventricular pressure and/or volume overload. In all the above situations in the presence of a left ventricle smaller than normal a very exhaustive approach has been reviewed in the decision making process, taking in account the literature reports as well as the personal experience. In each patient with 'borderline left ventricle' the elements to be considered for the decision making process between uni- and bi-ventricular type of repair, or for less ideal options of management, are the following: morphometric and functional parameters, hemodynamic data, available surgical options, results of the personal and institutional experience.

Quantitative assessment of right ventricular function using doppler tissue imaging in fetuses with and without heart failure.

BACKGROUND: Previous reports have established the use of Doppler tissue imaging (DTI) for noninvasive assessment of ventricular function, but the technique has not been validated for diagnosis of fetal cardiac failure. OBJECTIVE: The purpose of this study was to assess right ventricular (RV) function in fetuses with heart failure using DTI. METHODS: In all, 43 fetuses (36 control, 7 heart failure) were assessed using pulsed Doppler echocardiography combined with DTI. RV peak myocardial velocities during early diastole (Ea), atrial contraction, and systole were measured; and tricuspid peak velocities during early diastole (E) and atrial contraction. The ratio of E/Ea was used as an index of filling pressure were measured. From DTI, a Doppler-derived index of combined systolic/diastolic myocardial performance (DTI-Tei index) was measured. RESULTS: Compared with control fetuses, the mean Ea was significantly lower and the mean E was significantly higher in fetuses with heart failure, although these parameters did overlap between the 2 groups. The mean RV myocardial wall-motion velocity during atrial contraction, ratio of Ea/RV myocardial wall-motion velocity during atrial contraction, and RV myocardial wall-motion velocity during systole did not differ between the 2 groups. Compared with control fetuses, the mean E/Ea was significantly higher (9.71 +/- 0.91 vs 6.20 +/- 0.97; P <.0001) and the mean DTI-Tei index was significantly greater (0.79 +/- 0.11 vs 0.55 +/- 0.05; P <.0001) in fetuses with heart failure. In addition, the DTI-Tei index z score was >2 in all fetuses with heart failure. CONCLUSIONS: This study demonstrated a clinically important application of DTI-derived tricuspid annular velocities in fetuses with heart failure. Although DTI velocities were not sufficiently sensitive to identify fetuses with heart failure versus control fetuses, DTI-Tei index and E/Ea were useful and sensitive indicators of global RV dysfunction.

ECG of the month. Fatigue in a 34-year-old woman. Sinus bradycardia, right axis deviation, and right ventricular enlargement.

This obese, diabetic patient gives a history of a heart operation performed through a median sternotomy in the first year of her life. She is acyanotic and has a soft left parasternal systolic murmur that increases with inspiration. Her electrocardiogram (ECG) is shown below.

Systolic and diastolic function of the fetal single left ventricle.

BACKGROUND: The functionally single fetal right ventricle demonstrates reduced longitudinal relative to circumferential contraction velocities and deformation, a pattern similar to the normal fetal left ventricle. Altered diastolic properties are also present, with greater reliance on atrial contraction for right ventricular filling. It is unknown whether the functionally single left ventricle (SLV) demonstrates similar altered deformation patterns and diastolic properties. METHODS: Echocardiograms from 29 fetuses with SLVs were retrospectively compared with those from 48 controls with appropriately grown left ventricles. Ventricular function was assessed using Velocity Vector Imaging velocity, tissue deformation, two-dimensional, and Doppler flow parameters. RESULTS: Fetuses with functionally SLVs showed no difference in peak global left ventricular longitudinal velocity or displacement or strain, while global radial displacement was increased (P < .001). The ratio of longitudinal to circumferential deformation was also no different from that in controls. The SLVs showed increased diameters (P < .001) with normal lengths. Mitral inflow peak E-wave (P < .05) and A-wave (P < .0001) velocities were increased, with a reduced E/A ratio (P < .001). A-wave inflow fraction was also increased (P < .05), with no change in A duration. Although ejection time was no different, inflow duration was increased (P < .01) and there was a trend toward reduction of the Tei index (P = .07). CONCLUSIONS: The functionally single fetal left ventricle shows comparable changes to the single right ventricle, with a more spherical morphology and greater reliance on atrial contraction for ventricular filling than in controls. However, in contrast to the single right ventricle, the SLV had a normal longitudinal to circumferential deformation ratio with enhanced early diastolic filling.

Test-retest variability of volumetric right ventricular measurements using real-time three-dimensional echocardiography.

BACKGROUND: Substantial variability in sequential echocardiographic right ventricular (RV) quantification may exist. Interobserver and intraobserver values are well known, but acquisition (test-retest) variability has been rarely assessed. The objective of this study was to determine the test-retest variability of sequential RV volume and ejection fraction (EF) measurements by real-time three-dimensional echocardiography in patients with congenital heart disease and healthy controls. METHODS: Twenty-eight participants (21 patients with congenital heart disease, seven healthy controls; mean age, 30 ± 14 years; 43% men) underwent a series of three echocardiographic studies. To obtain interobserver and intraobserver test-retest variability, two sonographers acquired sequential RV data sets in each participant during one outpatient visit. RV volumetric quantification was done using semiautomated three-dimensional border detection. The variability data were analyzed using correlation coefficients, Bland-Altman analysis, and coefficients of variation. RESULTS: Absolute mean differences for sequential intraobserver acquisitions were 12 ± 12 mL for end-diastolic volume, 7 ± 6 mL for end-systolic volume, and 4 ± 3% for EF. Interobserver and intraobserver test-retest variability, respectively, were 7% and 7% for RV end-diastolic volume, 14% and 7% for end-systolic volume, and 8% and 6% for EF. CONCLUSIONS: Good test-retest variability, besides the practical nature of real-time three-dimensional echocardiography for RV volume and EF assessment, makes it a valuable technique for serial follow-up. Although it may be challenging to diminish all factors that can influence echocardiographic examination for serial follow-up, standardization of RV size and functional measurements should be a goal to produce more interchangeable data.

Right ventricular plication in a neonate with an unguarded tricuspid valvar orifice.

Unguarded tricuspid orifice is a congenital cardiac malformation characterized by absence of the tricuspid valvar leaflets in the setting of a normal atrioventricular junction and obligatory severe tricuspid regurgitation. A neonate with such a lesion presented with severe cardiac failure. The right atrium and ventricle were grossly dilated, and there were small muscular ventricular septal defects. We plicated the right-sided structures to create space for adequate expansion of the lungs and left ventricular filling, proceeding to staged conversion to the Fontan circulation. Our experience shows that neonates with cardiac failure due to dilated right-sided structures can be stabilized by surgical plication.

Pulmonary valve replacement improves but does not normalize right ventricular mechanics in repaired congenital heart disease: a comparative assessment using velocity vector imaging.

BACKGROUND: This study evaluated regional right ventricular (RV) mechanics before and after pulmonary valve replacement (PVR) by ultrasonic speckle tracking technology using velocity vector imaging (VVI). METHODS: Fifty-eight patients who underwent PVR (May 1999 to August 2007) were included. Two-dimensional Doppler indices included qualitative pulmonary incompetence, RV outflow tract peak gradient, RV systolic pressure estimate, indexed RV area, and fractional area change; VVI regional indices included peak systolic and diastolic velocities, peak systolic strain, maximal longitudinal displacement, and times to peak measure from 6 RV segments. RESULTS: PVR was performed at a median age of 12.1 years with echocardiographic analysis at median intervals of 2.8 months before and 30 months after PVR. Peak velocities and displacement increased in all 6 RV segments after PVR; peak systolic strain did not improve consistently. All indices remained significantly lower compared with normal values. There was a significant decrease in the degree of pulmonary incompetence, RV outflow tract gradient, RV systolic pressure, and indexed RV area (23.5 vs. 17.8 cm(2)/m(2)), but no significant change in the percentage of fractional area change (28.8% vs. 29.6%). CONCLUSION: Regional RV mechanics using VVI in a relatively young cohort shows mild improvement after PVR, but the RV is not normalized despite physiologic improvement in loading conditions. This suggests intrinsic dysfunction or chronic myocardial injury that is nonmodifiable or requires earlier intervention to optimize physiology. VVI appears to be a potentially useful quantitative tool for follow-up evaluation of RV performance after congenital heart disease surgery.

Cardiac resynchronization therapy for adult congenital heart disease patients with a systemic right ventricle: analysis of feasibility and review of early experience.

AIMS: Patients with a systemic right ventricle (RV) frequently develop heart failure and may benefit from cardiac resynchronization therapy (CRT). We aimed to assess the proportion of unselected patients with a systemic RV eligible for CRT and to review available data on the effect of CRT in congenital heart disease patients. METHODS AND RESULTS: Adhering to criteria derived from landmark CRT trials, we determined the eligibility of patients with a systemic RV for CRT. Seventy-five transposition of the great arteries (TGA) patients (age 29.5+/-10.2 years) and 49 patients with congenitally corrected (cc) TGA (age 36.2+/-12.8 years) were studied. Full criteria for CRT were met in 4.0% of the TGA patients and 4.1% of the ccTGA patients. Including New York Heart Association class 2 patients, 9.3% of TGA and 6.1% of ccTGA patients were eligible for CRT. CONCLUSION: Four to 9% of unselected patients with a systemic RV appear to be potential candidates for CRT. Although large clinical studies are currently lacking, available data consistently demonstrate that CRT improves haemodynamics in congenital heart disease patients and warrants further investigation.

Balloon angioplasty of critical coarctation in a 970-gram premature infant.

We describe a 970-gram infant born prematurely at 28 weeks gestation. On the third day of life, she presented in heart failure with Shone's syndrome and severe coarctation of the aorta. Despite initiation of intravenous prostaglandins, she developed progressive severe biventricular dysfunction and acidosis. Balloon angioplasty of the coarctation was successfully performed on the fifth day of life, with a dramatic improvement in ventricular function. At seven weeks of age, after improvement of ventricular function, she underwent surgical resection of the coarctation and arch advancement. At six months of age she is thriving and developing normally.

Corrección uno y medio. Resultados en el Instituto Nacional de Cardiología "Ignacio Chávez" / One and half ventricle repair. Surgical results at the Instituto Nacional de Cardiología

La hipertensión pulmonar primaria (HPP) es una enfermedad rara, progresiva y fatal. Debido a que los signos y síntomas son inespecíficos, al momento del diagnóstico los pacientes se presentan con una enfermedad ya avanzada. En años recientes se han realizado avances importantes en el conocimiento de esta enfermedad. Una nueva nomenclatura ha sido propuesta, se ha definido mejor la patobiología, se han identificado nuevos factores de riesgo y las nuevas estrategias terapéuticas parecen estar cambiando la historia natural y el pronóstico de esta enfermedad.