A Clinical Study Of Cadiovascular Manifestaions Of Pheochromocytoma

Background Pheochromocytomas and paragangliomas (PH/PCL) are rare tumors, which can cause blood pressure alterations and cardiac injuries. Reports regarding changes of cardiac structure and function, of hemodynamics in PH/PCL patients are few. This is the first study to apply two dimensional speckle tracking echocardiography in the evaluation of a case series of PH/PGL patients.Methods Seventy-eight PH/PGL patients receiving medical attention in Peking Union Medical College Hospital from October2010to April2013and46healthy controls were included in this study. Conventional echocardiographic parameters, speckle tracking left ventricular(LV) longitudinal strain and strain rate parameters were compared between patients and controls. Prevalence and presentations of cardiovascular manifestations including blood pressure abnormalities and cardiac injuries among patients were recorded. Clinical presentations, echocardiographic parameters, intraoperative hemodynamic changes, fluid and inotropic support regimes were compared among subgroups of patients with or without LV hypertrophy or left ventricular longitudinal strain abnormalities.Results1. Of the78patients,66(84.6%) were hypertensive,3(3.8%) experienced hypotensive episodes,45(57.7%) had histories indictive of cardiac injuries, including3(3.8%) with acute left-sided heart failure episodes,6(7.7%) with concurrent evolving cardiac enzyme elevation and alteration of ECG ST-T segment,6(7.7%) with decreased left ventricular ejection fraction(LVEF),5of which restored during follow-up,30(38.5%) with arrhythmias,25(32.1%) with LV hypertrophy as evidened by echocardiographic parameters.2. Among hypertensive PH/PGL patients, levels of24-hour urinary norepinephrine (435[61-766] vs.110[35-242]μg/24h, p=0.006) and epinephrine(3.51[3.01-4.53] vs.2.88[2.32-3.89] μg/24h, p=0.016) were significantly higher in LV hypertrophic group than in normal geometry group, while the duration, manifestation (sustained vs. paroxysmal) and degree of blood pressure elevation did not differ between the two groups.3. LVEF(75[70-79] vs.72[65-75]%, p=0.002), cardiac output index(3.30±0.75vs. 2.80±0.24L/min.m2, p<0.001), left ventricular mass index(LVMI)(56.9[41.1-109.5] vs.46.9[41.1-57.0] g/m2, p=0.002), left atrial volume index(LAVI)(25.62±7.02vs.19.03±5.07mL/m2, p<0.001) and mitral valve(MV) E/E’(average) ratio (8.42±3.93vs.6.46±2.44, p=0.003) in78PH/PGL patients was significantly higher than in46healthy controls. MV lateral E’ velocity (11.72±3.34vs.13.50±4.32cm/s, p=0.012) was significantly lower in patients than in controls.4. LV hypertrophy was associated with higher heart rate(93.4±20.6vs.77.3±15.8bpm, p=0.001), more prevalent arrhythmias(16in25vs.14in53, p=0.001), acute left-sided heart failure episodes(3in25vs.0in53, p=0.030), sustained LVEF depression(3in25vs.0in53, p=0.030) and ECG ST-T segment alterations in PH/PGL patients.5. Comparing with16healthy control, LV peak systolic longitudinal strain was significantly lower in78PH/PGL patients(-18.5±3.8vs.-21.6±1.2%, p<0.001) as well as75PH/PGL patients with preserved LVEF (-18.9±3.3vs.-21.6±1.2%, p<0.001).6. PH/PGL patients with normal LV geometry had significantly higher peak systolic longitudinal strain than patients with LV hypertrophy(-20.0±2.7vs.-15.3±3.9%, p<0.001), and significantly lower value than healthy controls(-20.0±2.7vs.21.6±1.2%, p=0.001).7. In56patients undergoing surgical resection of the tumors,9patients with abnormal longitidinal strain required significantly higher net fluid intake(3200[2700-4700] vs.2550[1863-3300]ml, p=0.037) than47patients with normal longitudinal strain, while the blood loss and duration of surgeries of the two groups did not differ significantly.Conclusions1. PH/PGL can cause blood pressure alterations, arrhythmias, and cardiomyopathies. Decreased LVEF, often reversible, occurs occasionally.2. Catecholamine is a causal factor of LV hypertrophy in PH/PGL patients independent of hypertension.3. LV hypertrophy in PH/PGL patients is associated with arrhythmias, acute left-sided heart failure, LV diastolic dysfunction and elevated LV filling pressure.4. Subclinical LV systolic dysfunction is present in certain PH/PGL patients with preserved LV ejeciton fraction.5. LV longitudinal strain abnormalities in PH/PGL patients is associated with LV hypertrophy and the need for more rigourous fluid regiems during surgery.