Paediatric sickle cell disease: pulmonary hypertension but normal vascular resistance.

BACKGROUND: Adults with sickle cell disease (SCD) and pulmonary hypertension have high mortality but death in SCD children with pulmonary hypertension is rare. The authors hypothesised that pulmonary hypertension in SCD children may be secondary to anaemia-induced high cardiac output rather than pulmonary vascular disease. METHODS: Two independent, validated techniques were used to estimate pulmonary vascular resistance (PVR) in 50 SCD children and 50 matched controls. Tricuspid regurgitant jet velocity (TRV) and right ventricular outflow tract velocity time integral were measured using Doppler echocardiography; PVR was calculated from their ratio. Acetylene rebreathing technique using respiratory mass spectrometry was also performed to calculate pulmonary blood flow and stroke index, an estimate of PVR. RESULTS: TRV was higher in SCD children compared with controls (2.28 vs 2.14 m/s, p=0.02). Fifteen of 34 (44%) children with haemoglobin of the SS genotype (HbSS) versus 1/16 (6%) children with haemoglobin of the SC genotype (HbSC) had pulmonary hypertension (TRV≥2.5 m/s) (p=0.009). Right ventricular stroke volume was higher (p<0.05) and Doppler PVR lower (1.20 (0.19) vs 1.31 (0.20) Wood units, p=0.04) in SCD children with pulmonary hypertension compared with controls. Qpeff and stroke index were higher in SCD children compared with controls (p<0.001 for both) and correlated with anaemia (p<0.001) and TRV (p=0.03). There was no correlation between TRV and history of asthma or acute chest syndrome. CONCLUSIONS: Pulmonary hypertension due to raised cardiac output is common in HbSS SCD children and is associated with normal PVR. PVR should be measured before therapy with agents such as sildenafil or bosentan is contemplated.

Usefulness of changes in left ventricular wall thickness to predict full or partial pressure reperfusion in ST-elevation acute myocardial infarction.

Experimental studies have shown that if an acute transmural myocardial infarction is reperfused at full pressure there is an immediate and persisting increase in end-diastolic wall thickness (EDWT) due to massive intramural edema, with the amount of edema inversely related to the residual stenosis in the infarct-related artery. This study investigated if these findings are paralleled in the clinical setting and whether the resultant myocardial substrate differs after percutaneous coronary intervention (PCI) versus thrombolysis (the latter having a higher incidence of residual flow limiting stenosis in the culprit vessel). Eighty-eight consecutive patients with ST-elevation myocardial infarction were enrolled. Twenty-seven patients underwent primary PCI, 23 had rescue PCI, and 38 had thrombolysis. Standard M-mode and 2-dimensional echocardiographies were performed within 12 hours. Regional EDWT was measured in 904 infarct-related segments after the different reperfusion strategies and compared with 504 remote noninfarcted segments. EDWT of infarct-related segments after primary PCI was significantly increased compared with normal segments. At follow-up, after 6 months, EDWT of these segments was significantly decreased, indicating transmural infarction. EDWT of infarct-related segments after thrombolysis did not differ from that of normal segments. After rescue PCI, EDWT of infarct-related segments was significantly decreased compared with that of normal segments. In conclusion, full-pressure restoration of epicardial blood flow after transmural myocardial infarction causes an immediate increase in EDWT, easily detected by echocardiography. In contrast, pressure-limiting reperfusion (typical for thrombolysis) resultsin normal EDWT. This confirms experimental data that PCI and thrombolysis can differ in their resultant myocardial substrate.

Changes in systolic left ventricular function in isolated mitral regurgitation. A strain rate imaging study.

AIMS: The aim of the present study is to understand the changes in left ventricular (LV) regional systolic deformation based on strain rate (SR) imaging in patients with isolated mitral regurgitation (MR). Progressive LV dilatation and irreversible myocardial damage as a result of chronic isolated MR are important causes of morbidity and mortality in patients following valve surgery. To date, there is no specific diagnostic method to detect subclinical changes in systolic function before irreversible dysfunction occurs. METHODS AND RESULTS: Seventy-seven individuals were studied: 54 asymptomatic patients (age 56 +/- 12) with isolated non-ischaemic MR divided into three groups: mild, moderate, and severe and 23 healthy subjects. All underwent a standard echo examination and a tissue Doppler study. A mathematical study was carried out to predict how SR should alter with increasing dimensions and due to irreversible myocardial damage. Radial as well as longitudinal peak systolic SR was significantly decreased in patients with severe MR compared to the other groups (LV posterior wall: P = 0.0006, septum: P = 0.0004, LV lateral wall: P = 0.0003). From both modelling and in our patients, deformation correlated inversely with LV end-diastolic diameter and end-systolic diameter (ESD). Deformation measurements (corrected for increased geometry) enabled the identification of patients classically referred to as at risk of irreversible myocardial damage (ESD > or = 4.5 cm). CONCLUSION: In patients with a wide range of MR, deformation remains unchanged due to a balance of increased dimensions and increased stroke volume. Only when contractility is expected to change, deformation will significantly decrease. SR imaging indices, corrected for geometry, might potentially be useful in detecting subclinical deterioration in LV function in asymptomatic patients with severe MR.