permissão concedida pela Elsevier para reprodução do material em português somente. Abreviações: 2D: bidimensional 3D: tridimensional Área A: área abaixo da curva da onda A da fluxo mitral Onda A: Fluxo mitral correspondente ao momento da contração atrial Onda a': Onda obtida no nível do anel mitral pelo Doppler tecidual durante a contração atrial Onda Ar: Velocidade de fluxo reverso pulmonar ou sistêmico durante a contração atrial VAo: Valva aórtica ASE: Sociedade Americana de Ecocardiografia AV: Atrioventricular SC: Superfície corpórea CC: Cardiopatia congênita Onda D: Componente diastólico do fluxo venoso pulmonar ou sistêmico dP/dt: Primeira derivado de pressão em relação ao tempo Área A: Área abaixo da curva da onda A do fluxo mitral (diástole precoce) Onda E: Fluxo mitral correspondente ao componente diastólico precoce Onda e': Onda obtida pelo Doppler tecidual no nível do anel valvar mitral durante a diástole precoce DDF: Diâmetro diastólico final VDF: Volume diastólico final FE: Fração de ejeção DSF: Diâmetro sistólico final VSF: Volume sistólico final VCI: Veia cava inferior TCIV': Tempo de contração isovolumétrica obtido ao Doppler tecidual TRIV: Tempo de relaxamento isovolumétrico obtido ao Doppler convencional TRIV': Tempo de relaxamento isovolumétrico obtido ao Doppler tecidual L: Comprimento AE: Átrio esquerdo VE: Ventrículo esquerdo RNM: Ressonância nuclear magnética VM: Valva mitral PFR SV : Taxa máxima de enchimento/volume ejetado VP: valva pulmonar AD: Átrio direito VD: Ventrículo direito Onda s': Onda obtida pelo Doppler tecidual no nível do anel valvar mitral durante a sístole Onda S: Componente sistólico do fluxo venoso pulmonar ou sistêmico ▲D: Fração de encurtamento TAPSE: Deslocamento do anel tricúspide durante a sístole VT: Valva tricúspide V: Volume ventricular VTI: Integral da velocidade x tempo

No abstract

It has been 50 years since Francis Fontan pioneered the operation that today bears his name. Initially designed for patients with tricuspid atresia, this procedure is now offered for a vast array of congenital cardiac lesions when a circulation with 2 ventricles cannot be achieved. As a result of technical advances and improvements in patient selection and perioperative management, survival has steadily increased, and it is estimated that patients operated on today may hope for a 30-year survival of >80%. Up to 70 000 patients may be alive worldwide today with Fontan circulation, and this population is expected to double in the next 20 years. In the absence of a subpulmonary ventricle, Fontan circulation is characterized by chronically elevated systemic venous pressures and decreased cardiac output. The addition of this acquired abnormal circulation to innate abnormalities associated with single-ventricle congenital heart disease exposes these patients to a variety of complications. Circulatory failure, ventricular dysfunction, atrioventricular valve regurgitation, arrhythmia, protein-losing enteropathy, and plastic bronchitis are potential complications of the Fontan circulation. Abnormalities in body composition, bone structure, and growth have been detected. Liver fibrosis and renal dysfunction are common and may progress over time. Cognitive, neuropsychological, and behavioral deficits are highly prevalent. As a testimony to the success of the current strategy of care, the proportion of adults with Fontan circulation is increasing. Healthcare providers are ill-prepared to tackle these challenges, as well as specific needs such as contraception and pregnancy in female patients. The role of therapies such as cardiovascular drugs to prevent and treat complications, heart transplantation, and mechanical circulatory support remains undetermined. There is a clear need for consensus on how best to follow up patients with Fontan circulation and to treat their complications. This American Heart Association statement summarizes the current state of knowledge on the Fontan circulation and its consequences. A proposed surveillance testing toolkit provides recommendations for a range of acceptable approaches to follow-up care for the patient with Fontan circulation. Gaps in knowledge and areas for future focus of investigation are highlighted, with the objective of laying the groundwork for creating a normal quality and duration of life for these unique individuals.

Rationale: Pulmonary hypertension (PH) is associated with poor outcomes among preterm infants with bronchopulmonary dysplasia (BPD), but whether early signs of pulmonary vascular disease are associated with the subsequent development of BPD or PH at 36 weeks post-menstrual age (PMA) is unknown.Objectives: To prospectively evaluate the relationship of early echocardiogram signs of pulmonary vascular disease in preterm infants to the subsequent development of BPD and late PH (at 36 wk PMA).Methods: Prospectively enrolled preterm infants with birthweights 500-1,250 g underwent echocardiogram evaluations at 7 days of age (early) and 36 weeks PMA (late). Clinical and echocardiographic data were analyzed to identify early risk factors for BPD and late PH. Measurements and Main Results:A total of 277 preterm infants completed echocardiogram and BPD assessments at 36 weeks PMA. The median gestational age at birth and birthweight of the infants were 27 weeks and 909 g, respectively. Early PH was identified in 42% of infants, and 14% were diagnosed with late PH. Early PH was a risk factor for increased BPD severity (relative risk, 1.12; 95% confidence interval, 1.03-1.23) and late PH (relative risk, 2.85; 95% confidence interval, 1.28-6.33). Infants with late PH had greater duration of oxygen therapy and increased mortality in the first year of life (P , 0.05).Conclusions: Early pulmonary vascular disease is associated with the development of BPD and with late PH in preterm infants. Echocardiograms at 7 days of age may be a useful tool to identify infants at high risk for BPD and PH.Keywords: bronchopulmonary dysplasia; pulmonary vascular disease; pulmonary hypertension; echocardiography; prematurity At a Glance CommentaryScientific Knowledge on the Subject: Preterm infants remain at high risk for late respiratory morbidity and mortality caused by the development of bronchopulmonary dysplasia (BPD) and pulmonary hypertension (PH). Early injury to the developing lung can impair angiogenesis and alveolarization and result in simplification of distal lung airspace and the clinical manifestations of BPD and PH. However, whether early signs of pulmonary vascular disease are indicative of the subsequent development of BPD or PH at 36 weeks postmenstrual age (PMA) has not been well established.What This Study Adds to the Field: This paper presents a longitudinal study identifying echocardiogram-derived risk factors at 7 days of age for the subsequent development of both BPD and PH. We also describe the incidence of PH at 36 weeks PMA and its relationship to BPD severity.

As used in clinical practice, echocardiography often identifies pulmonary hypertension in young children with chronic lung disease; however, estimates of systolic pulmonary artery pressure were not obtained consistently and were not reliable for determining the severity of pulmonary hypertension.

Background-Left ventricular (LV) torsional deformation, based in part on the helical myocardial fiber architecture, is an important component of LV systolic and diastolic performance. However, there is no comprehensive study describing its normal development during childhood and adult life. Methods and Results-Forty-five normal subjects (25 children and 20 adults; aged 9 days to 49 years; divided into 5 groups: infants, children, adolescents, and young and middle-age adults) underwent assessment of LV torsion and untwisting rate by Doppler tissue imaging. LV torsion increased with age, primarily owing to augmentation in basal clockwise rotation during childhood and apical counterclockwise rotation during adulthood. Although LV torsion and untwisting overall showed age-related increases, when normalized by LV length, they showed higher values in infancy and middle age. The proportion of untwisting during isovolumic relaxation was lowest in infancy, increased during childhood, and leveled off thereafter, whereas peak untwisting performance (peak untwisting velocity normalized by peak LV torsion) showed a decrease during adulthood. Conclusions-We have shown the maturational process of LV torsion in normal subjects. Net LV torsion increases gradually from infancy to adulthood, but the determinants of this were different in the 2 age groups. The smaller LV isovolumic untwisting recoil during infancy and its decline in adulthood may suggest mechanisms for alterations in diastolic function.

No abstract

Background Pulmonary hypertension (PH) increases right ventricular (RV) pressure, resulting in septal shift and RV dilation. Few echocardiographic measures have been used to evaluate severity and outcomes in children with PH. The aims of this study were to compare the RV to left ventricular (LV) diameter ratio at end-systole (RV/LV ratio) in normal controls and patients with PH, to correlate the RV/LV ratio with invasive hemodynamic measures, and to evaluate its association with outcomes in children with PH. Methods The RV/LV ratio was compared retrospectively between 80 matched normal controls and 84 PH patients without shunts. Of the patients with PH, 49 children underwent 94 echocardiographic studies and cardiac catheterizations within 48 hours (13 patients had simultaneous measurements). The RV/LV ratio was correlated against hemodynamic measures. Kaplan-Meier curves and a Cox proportional-hazards regression model were used to assess relationships between RV/LV ratio and time until an adverse clinical event (initiation of intravenous prostacyclin therapy, atrial septostomy, death, or transplantation). Results RV/LV ratios were lower in controls compared with patients with PH (mean, 0.51 [95% confidence interval, 0.48–0.54] vs 1.47 [95% confidence interval, 1.25–1.70], P < .01). The RV/LV ratio correlated significantly with mean pulmonary artery pressure, systolic pulmonary artery pressure, systolic pulmonary artery pressure as a percentage of systemic pressure, and pulmonary vascular resistance index (r = 0.65 [P < .01], r = 0.6 [P < .01], r = 0.49 [P < .01], and r = 0.43 [P < .01], respectively). Twenty-two patients with PH with RV/LV ratios > 1 had adverse events within a median of 1.1 years from their earliest echocardiographic studies. Increasing RV/LV ratio was associated with an increasing hazard for a clinical event (hazard ratio, 2.49; 95% confidence interval, 1.92–3.24). Conclusions The RV/LV end-systolic diameter ratio can easily be obtained noninvasively in the clinical setting and can be used in the management of patients with PH. The RV/LV ratio incorporates both pathologic septal shift and RV dilation in children with PH and correlates with invasive measures of PH. An RV/LV ratio > 1 is associated with adverse clinical events.