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Ricardo H Pignatelli

Publications and source records attributed to Ricardo H Pignatelli.

12 recordsLinked to original sources
PubMed2006

Left ventricular non-compaction cardiomyopathy in children: characterisation of clinical status using tissue Doppler-derived indices of left ventricular diastolic relaxation.

BACKGROUND: Left ventricular non-compaction (LVNC) may manifest an undulating phenotype ranging from dilated to hypertrophic appearance. It is unknown whether tissue Doppler (TD) velocities can predict adverse clinical outcomes including death and need for transplantation in children with LVNC. METHODS AND RESULTS: 56 children (median age 4.5 years, median follow-up 26 months) with LVNC evaluated at one hospital from January 1999 to May 2004 were compared with 56 age/sex-matched controls. Children with LVNC had significantly decreased early diastolic TD velocities (Ea) at the lateral mitral (11.0 vs 17.0 cm/s) and septal (8.9 vs 11.0 cm/s) annuli compared with normal controls (p<0.001 for each comparison). Using receiver operator characteristic curves, the lateral mitral Ea velocity proved the most sensitive and specific predictor for meeting the primary end point (PEP) at 1 year after diagnosis (area under the curve = 0.888, SE = 0.048, 95% CI 0.775 to 0.956). A lateral mitral Ea cut-off velocity of 7.8 cm/s had a sensitivity of 87% and a specificity of 79% for the PEP. Freedom from death or transplantation was 85% at 1 year and 77% at 2 years. CONCLUSIONS: TD velocities are significantly reduced in patients with LVNC compared with normal controls. Reduced lateral mitral Ea velocity helps predict children with LVNC who are at risk of adverse clinical outcomes including death and need for cardiac transplantation.

Adolescent↗
PubMed2006

Rapid ventricular remodeling with left ventricular unloading postventricular assist device placement: new insights with strain imaging.

We report the case of a 6-year old girl who presented with severe dilated cardiomyopathy. Cross-sectional echocardiography demonstrated a severely dilated hypokinetic left ventricle with an ejection fraction less than 10%. She developed intractable ventricular tachyarrhythmias and subsequently had a left ventricular assist device implanted. Within 1 week of mechanical support, echocardiography demonstrated dramatic reverse remodeling of the left ventricle with marked improvement in myocardial deformation using strain rate imaging. This report further highlights the potential for rapid remodeling after mechanical support of the failing myocardium.

Cardiomyopathy, Dilated↗
PubMed2005

Characteristics and outcomes of fetuses with pericardial effusions.

Little is known about the characteristics and outcomes of fetuses with pericardial effusions (PEs); therefore, this study sought to identify factors associated with fetal PEs and the natural histories and outcomes of fetuses with PEs. Large PEs are associated with a greater likelihood of structural heart disease, impaired cardiac function, and chromosomal abnormalities, and PEs with hydrops or extracardiac malformations are associated with death. Most fetal PEs resolve, and fetuses with isolated PEs have a very good prognosis.

Adolescent↗
PubMed2005

Usefulness of real-time navigator magnetic resonance imaging for evaluating coronary artery origins in pediatric patients.

Navigator coronary magnetic resonance imaging (MRI) was evaluated in assessing coronary artery origins in a pediatric and adolescent population. Sixty-five consecutive infants, children, or adolescents (age range 11 days to 21 years) were referred for MRI evaluations to assess coronary artery origins. Coronary artery origins were unambiguously delineated in 62 of 65 patients. In 3 patients, irregular arrhythmias precluded cardiac gating of the magnetic resonance acquisition. Two patients had anomalous coronary artery origins detected. Twenty-six patients required sedation for the studies. Free-breathing 3-dimensional MRI with real-time navigator correction is a robust method for delineating the coronary artery origins in pediatric and adolescent patients.

Adolescent↗
PubMed2005

Transcatheter atrial septal defect closure: modified balloon sizing technique to avoid overstretching the defect and oversizing the Amplatzer septal occluder.

The objective of this study was to evaluate a new technique of sizing atrial septal defects (ASDs) for transcatheter device closure. ASD closure using the Amplatzer septal occluder (ASO) device is commonly performed. Complications, including arrhythmias, pericardial effusions, and perforations, may be related to oversizing ASDs and choosing larger devices. Two methods were used to size ASDs using a compliant balloon. In some patients, the balloon was inflated until a waist was visible [(+)waist]; in others, only until no shunting was demonstrable by echocardiogram [echo; (-)waist]. The device was selected and implanted using standard procedure and echo guidance. One hundred seventeen patients underwent secundum ASD closure with an ASO device. There were 43 patients in the (-)waist group and 74 in the (+)waist group. All devices were implanted successfully. The initial echo ASD diameter was larger in the (-)waist group compared to the (+)waist group (P = 0.01). There was a smaller difference between the initial echo and balloon-sized ASD diameters in the (-)waist group (P < 0.02). ASO device size implanted (in mm greater than echo ASD diameter) was smaller in the (-)waist group (P < 0.01). There were 0/43 complications in the (-)waist group and 5/74 in the (+)waist group. The complete closure rate was the same in both groups. Sizing an ASD by inflating a compliant balloon just until shunting is eliminated, and not until a waist is visible, results in less overstretching of the ASD and selection of a smaller ASO device, achieving similar closure rates and potentially fewer complications.

Adolescent↗
PubMed2005

Impact of chronic left ventricular preload and afterload on Doppler tissue imaging velocities: a study in congenital heart disease.

BACKGROUND: Doppler tissue imaging (DTI) velocities have been reported to be relatively independent of changes in ventricular loading conditions in adult studies. The clinical impact of altered left ventricular (LV) preload and afterload on DTI velocities in children with congenital heart disease has not been adequately evaluated. The purpose of this study was to evaluate the impact of chronic LV preload and afterload on DTI velocities in children with isolated ventricular septal defect and aortic valve stenosis compared with age-matched normal and abnormal (dilated cardiomyopathy) control groups. METHODS: From an apical 4-chamber view, DTI velocities were obtained at the cardiac base at the lateral mitral annulus, lateral tricuspid annulus, and interventricular septum in early diastole, late diastole, and ventricular systole. RESULTS: The majority of DTI velocities did not change significantly in patients with increased LV preload. Patients with increased LV afterload had significantly decreased systolic and early diastolic DTI velocities at both the lateral mitral annulus and ventricular septum compared with control subjects. Children with dilated cardiomyopathy had significantly decreased DTI velocities at all myocardial annular locations. CONCLUSIONS: We conclude that increases in chronic LV preload do not significantly affect the majority of DTI velocities in children with ventricular septal defects. In addition, significantly increased chronic LV afterload in children with aortic valve stenosis is associated with decreased DTI velocities in the absence of other identifiable abnormalities of LV function. Decreased DTI velocity may be secondary to increased afterload or may alternatively be an early marker of subclinical LV longitudinal dysfunction.

Adolescent↗
PubMed2004

Characterization of left ventricular diastolic function by tissue Doppler imaging and clinical status in children with hypertrophic cardiomyopathy.

BACKGROUND: Conventional transmitral Doppler indices are unreliable in assessing clinical status in patients with hypertrophic cardiomyopathy (HCM) because they are affected by loading conditions. This study sought to determine whether tissue Doppler velocities are predictive of adverse clinical outcomes including death, cardiac arrest, ventricular tachycardia (VT), significant cardiac symptoms, and exercise capacity in children with HCM. METHODS AND RESULTS: We studied 80 consecutive children (median age 12 years, median follow-up 26 months) evaluated at 1 hospital from January 1999 to August 2003 compared with 80 age- and gender-matched controls. Patients underwent echocardiography, ambulatory Holter monitoring, and upright exercise testing. Children with HCM had significantly decreased early diastolic tissue Doppler velocities at the lateral mitral (13.2 versus 19.3 cm/s), tricuspid (13.3 versus 16.3 cm/s), and septal (9.4 versus 13.5 cm/s) annuli compared with controls (P<0.001 for each comparison). By forward stepwise regression analysis, early transmitral left ventricular filling velocity (E)/septal Ea ratio predicted death, cardiac arrest, or VT (r=0.610, R2=0.37, P<0.001). Peak oxygen consumption (VO2) was most predictive of children who developed symptoms (r=0.427, R2=0.182, P<0.001). Peak VO2 correlated inversely with E/Ea septal ratio (r=-0.740, P<0.01). CONCLUSIONS: Transmitral E/septal Ea ratio predicts children with HCM who are at risk of adverse clinical outcomes including death, cardiac arrest, VT, and significant cardiac symptoms. Peak VO2 correlated with peak exercise capacity in HCM patients.

Adolescent↗
PubMed2004

Impact of cardiac growth on Doppler tissue imaging velocities: a study in healthy children.

BACKGROUND: Doppler tissue imaging (DTI) is a useful modality to quantitatively assess regional myocardial function. Studies attempting to establish reference values for DTI velocities in healthy children have been limited by small sample sizes and limited age distribution. In addition, the clinical effect of cardiac growth and other demographic and echocardiographic parameters on DTI velocities during childhood has not been adequately evaluated. METHODS: Pulsed wave DTI velocities were obtained in 325 healthy children at the lateral mitral annulus, interventricular septum, and lateral tricuspid annulus during early diastole, late diastole, and ventricular systole and were compared with demographic and echocardiographic study variables. RESULTS: In healthy children, parameters of cardiac growth, most notably left ventricular end-diastolic dimension, have the most significant correlation with the majority of DTI velocities. Age was also significantly correlated with most DTI velocities whereas sex, heart rate, and other echocardiographic parameters demonstrated minimal or no correlation. CONCLUSIONS: This study establishes reference values for DTI velocities and demonstrates the important clinical effects of cardiac growth and age on DTI velocities in neonates and children.

Adolescent↗
PubMed2003

Clinical characterization of left ventricular noncompaction in children: a relatively common form of cardiomyopathy.

BACKGROUND: Left ventricular noncompaction (LVNC) is a reportedly uncommon genetic disorder of endocardial morphogenesis with a reportedly high mortality rate. The purpose of this study was to identify the clinical characteristics of children with LVNC. METHODS AND RESULTS: We retrospectively reviewed 36 children with LVNC evaluated at Texas Children's Hospital (TCH) from January 1997 to December 2002. Five children had associated cardiac lesions. There were 16 girls and 20 boys. The median age at presentation was 90 days (range, 1 day to 17 years). The median duration of follow-up was 3.2 years (range, 0.5 to 12 years). Twenty-seven patients (75%) had ECG abnormalities, most commonly biventricular hypertrophy (10 patients, 28%). Both ventricles were involved in 8 patients (22%) and only the left ventricle in 28 patients (78%). Left ventricular systolic function was depressed in 30 patients (83%), with a median ejection fraction of 30% (range, 15% to 66%) at diagnosis. Nine patients presenting in the first year of life with depressed left ventricular contractility had a transient recovery of function; however, ejection fraction deteriorated later in life, at a median interval of 6.3years (range, 3 to 12 years). Two patients had an "undulating" phenotype from dilated to hypertrophic cardiomyopathy. Two patients (6%) were identified with an underlying G4.5 gene mutation. Five patients (14%) died during the study. CONCLUSIONS: LVNC does not have an invariably fatal course when diagnosed in the neonatal period. A significant number of patients have transient recovery of function followed by later deterioration, which may account for many patients presenting as adults, some manifesting an "undulating" phenotype.

Acyltransferases↗
PubMed2003

Echocardiographic presentations of endocarditis, and risk factors for rupture of a sinus of Valsalva in childhood.

BACKGROUND: In recent years, the diagnosis of infective endocarditis has been enhanced by the use of echocardiography. We sought, therefore, to review its effect on the management of endocarditis in children. METHODS: We reviewed all the patients presenting to our institution for evaluation for infective endocarditis from May 1994 to January 2002. The patients were stratified according to whether or not they had congenitally malformed hearts. RESULTS: Of the 90 referred patients identified, 46 (51%) had positive ultrasonic findings. Of these, we excluded 26 patients because of the presence of indwelling lines. The remaining 20 patients with features of endocarditis had a median age of 6.5 years, and a range from 0.14 to 8.5 years. There were 4 patients with normal hearts, and 16 with congenital cardiac malformations. We identified rupture of a sinus of Valsalva in four patients, with rupture into the left ventricle in two, and into the right ventricle and right atrium in one each. The mitral valve was involved in six patients, the aortic valve in another six, including all four with rupture of the sinus of Valsalva, both mitral and aortic valves in three, the pulmonary trunk in three patients, and the tricuspid valve and a Blalock-Taussig shunt in one patient each. Organisms isolated included Streptococcus mitis in 4 patients, Streptococcus pneumoniae in 2 patients, Streptococcus sanguis in 1, Staphylococcus aureus in 3, Staphylococcus epidermidis in 1, and Enteroccocus in 2. Cultures proved negative in 7 patients. Surgical intervention was needed in 12 patients, and one died (5%). Only the left-sided chambers were involved in those with normal hearts. Both patients infected with Streptococcus pneumoniae had rupture of a sinus of Valsalva. CONCLUSION: Involvement of the left-sided chambers is more likely in structurally normal hearts, and in cases with rupture of a sinus of Valsalva, in which case infection with Streptococcus pneumonia should be suspected.

Aortic Rupture↗
PubMed2003

Role of echocardiography versus MRI for the diagnosis of congenital heart disease.

During the last few decades, significant strides have been made in the field of noninvasive imaging for the patient with congenital heart disease. Echocardiography and MRI continue to provide improved means of anatomic and functional assessment in children and adults with congenital heart lesions. This review reports some of the recent advances in tissue Doppler, strain rate, and integrated backscatter, and highlights exciting current and future potential developments in their application. We also discuss advances in MR in evaluation of cardiac anatomy and function in congenital heart disease.

Adult↗