ABSTRACT
AIM: This study determined cardiovascular impairment in young children with obstructive respiratory disease who were assessed using the opening interrupter technique (RINT). METHODS: This pilot study enrolled 41 children who had been referred to pulmonology and allergology specialists at the University of Catania, Italy, from March to July 2017: 23 (mean age 4.13 ± 0.62 years) had chronic coughs and wheezing and 18 controls (mean age 4.27 ± 0.66 years) had obstructive chest disease, but were otherwise healthy. Airway resistance was evaluated using RINT and cardiac function by studying the ejection fraction, pulmonary artery systolic pressure (PASP), tricuspid annular plane systolic excursion and tricuspid flow propagation velocity (TFPV). RESULTS: The RINT and PASP values were significantly higher in the patient group when compared to the controls, but the TFPV values were lower. A direct and significant Spearman's correlation coefficient (r) between RINT and PASP values was observed (r = 0.81). We found a significant inverse correlation between RINT and TFPV (r = -0.83), as well as TFPV and PASP (r = -0.78). CONCLUSION: This study showed that children with obstructive respiratory diseases had a major risk of cardiovascular impairment. Impaired diastolic function of the right ventricle occurred very early when airway resistance was abnormally increased.
Subject(s)
Diastole , Respiration Disorders/complications , Respiration Disorders/physiopathology , Ventricular Dysfunction, Right/etiology , Airway Resistance , Child, Preschool , Cohort Studies , Female , Humans , Male , Pilot ProjectsABSTRACT
BACKGROUND: Enzyme replacement therapy (ERT) for infantile-onset Pompe disease has been commercially available for almost 10 years. We report the experience of its use in a cohort treated at three specialist lysosomal treatment centres in the UK. METHODS: A retrospective case-note review was performed, with additional data being gathered from two national audits on all such patients treated with ERT. The impact on the outcome of various characteristics, measured just prior to the initiation of ERT (baseline), was evaluated using logistic regression. RESULTS: Thirty-three patients were identified; 13/29 (45%) were cross-reactive immunological material (CRIM) negative, and nine were immunomodulated. At baseline assessment, 79% were in heart failure, 66% had failure to thrive and 70% had radiological signs of focal pulmonary collapse. The overall survival rate was 60%, ventilation-free survival was 40% and 30% of patients were ambulatory. Median follow-up of survivors was 4 years, 1.5 months (range 6 months to 13.5 years). As with previous studies, the CRIM status impacted on all outcome measures. However, in this cohort, baseline failure to thrive was related to death and lack of ambulation, and left ventricular dilatation was a risk factor for non-ventilator-free survival. CONCLUSION: The outcome of treated patients remains heterogeneous despite attempts at immunomodulation. Failure to thrive at baseline and left ventricular dilation appear to be associated with poorer outcomes.
Subject(s)
Glycogen Storage Disease Type II/drug therapy , Glycogen Storage Disease Type II/mortality , Cardiomyopathies/metabolism , Cardiomyopathies/mortality , Cross Reactions , Enzyme Replacement Therapy/methods , Female , Glycogen Storage Disease Type II/metabolism , Humans , Infant , Lysosomes/metabolism , Male , Retrospective Studies , Survival Rate , United Kingdom , Ventricular Dysfunction, Left/metabolism , Ventricular Dysfunction, Left/mortalityABSTRACT
OBJECTIVE: To measure the left and right ventricular myocardial velocities using tissue Doppler imaging (TDI) in the first 24 h of life in neonates. DESIGN: Left and right ventricular peak systolic (S'), early diastolic (E') and late diastolic (A') myocardial velocities were measured using TDI alongside standard echocardiography (including peak diastolic atrioventricular flow, E). E/E' ratio was calculated for both ventricles. SETTING: UK neonatal intensive care unit. PATIENTS: 43 neonates were prospectively recruited into three groups: term (n=16), preterm (30-36 weeks, n=12) and very preterm (<30 weeks, n=15). RESULTS: Myocardial velocities increased with increasing gestation. Right ventricular velocities were significantly greater than left. E/E' ratio decreased with increasing gestation. Left E/E' ratio was higher than right in each group. CONCLUSIONS: TDI is feasible in preterm neonates and enables the acquisition of myocardial velocities. With increasing gestation, higher myocardial velocities and lower E/E' ratios were found. The addition of TDI to standard neonatal echocardiography may provide additional information about cardiac function.
Subject(s)
Infant, Newborn/physiology , Ventricular Function, Left/physiology , Ventricular Function, Right/physiology , Blood Flow Velocity/physiology , Echocardiography, Doppler/methods , Feasibility Studies , Female , Gestational Age , Humans , Infant, Premature , Intensive Care, Neonatal/methods , Male , Pilot Projects , Reproducibility of ResultsABSTRACT
UNLABELLED: Papillary fibroelastoma is a benign tumour of the cardiac valve apparatus. We present an unusual case of life-threatening pulmonary haemorrhage and disseminated intravascular coagulation in a neonate associated with this benign cardiac tumour. Papillary fibroelastoma of the tricuspid valve rarely presents in children and, to our knowledge, this is only the second reported case in a neonate. The patient was successfully managed by anticoagulation therapy followed by surgical excision of the tumour. This case illustrates the potentially fatal presentation of this benign cardiac tumour among neonates. CONCLUSION: Pulmonary haemorrhage of this degree is unusual in an otherwise healthy term neonate and needs careful investigation for unusual pathology including potential sources of pulmonary emboli in the heart. We emphasize the value of echocardiography in the evaluation of unexpected pulmonary haemorrhage in the newborn.
