Familial Primary Pulmonary Hypertension ; Humans ; Hypertension, Pulmonary ; Pulmonary Arterial Hypertension

Algorithms ; Familial Primary Pulmonary Hypertension ; Humans ; Hypertension, Pulmonary/diagnosis* ; Pulmonary Arterial Hypertension

Pulmonary hypertension is a hemodynamic and pathophysiological condition defined as an increase in mean pulmonary arterial pressure > or =25 mmHg at rest as assessed by right heart catheterization. Clinical conditions of pulmonary hypertension are classified into 6 clinical groups with different pathological, pathophysiological, prognostic and therapeutic features. The treatment strategy is remarkably different among 6 clinical groups. Group 1 (pulmonary arterial hypertension) is the only clinical group with specific drug therapy and an evidence-based treatment algorithm is provided. Following descriptions are about the clinical classification of pulmonary hypertension updated at 4th World symposium of pulmonary hypertension held in 2008.
Arterial Pressure ; Cardiac Catheterization ; Cardiac Catheters ; Hemodynamics ; Hypertension ; Hypertension, Pulmonary

Anastomosis, Surgical ; Child ; Familial Primary Pulmonary Hypertension ; Humans ; Hypertension, Pulmonary/surgery* ; Pulmonary Arterial Hypertension ; Pulmonary Artery/surgery*

In this study, we evaluated systemic and pulmonary hemodynamic changes with the administration of diltiazem into pulmonary artery in hypoxia(F1O2 0.15)-induced pulmonary vasoconstriction in 7 mongrel dogs. Hypoxic gas induced pulmonary vasoconstriction(HPV) resulted in 23.8% increase of mean pulmonary arterial pressure(PAP) without any changes of pulmonary vascular resistance(PVR). A 200 ug/kg intravenous bolus of diltazem, followed by 10 ug/kg/min(DL)and 15 ug/kg/min(DH) for 20 min, respectively, produced no change in pulmonary arterial pressure. But this was accompanied by increase in PVR and PVR/SVR ration in DL and 20 min after DH. We conclude that diltiazem does not seem to decrease acute hypoxic pulmonary vasoconstrition in dogs and it might not be beneficial drug for this kind of pulmoary hypertension.
Animals ; Arterial Pressure ; Diltiazem* ; Dogs* ; Hemodynamics ; Hypertension ; Pulmonary Artery ; Vasoconstriction*

In this study, we evaluated systemic and pulmonary hemodynamic changes with the administration of diltiazem into pulmonary artery in hypoxia(F1O2 0.15)-induced pulmonary vasoconstriction in 7 mongrel dogs. Hypoxic gas induced pulmonary vasoconstriction(HPV) resulted in 23.8% increase of mean pulmonary arterial pressure(PAP) without any changes of pulmonary vascular resistance(PVR). A 200 ug/kg intravenous bolus of diltazem, followed by 10 ug/kg/min(DL)and 15 ug/kg/min(DH) for 20 min, respectively, produced no change in pulmonary arterial pressure. But this was accompanied by increase in PVR and PVR/SVR ration in DL and 20 min after DH. We conclude that diltiazem does not seem to decrease acute hypoxic pulmonary vasoconstrition in dogs and it might not be beneficial drug for this kind of pulmoary hypertension.
Animals ; Arterial Pressure ; Diltiazem* ; Dogs* ; Hemodynamics ; Hypertension ; Pulmonary Artery ; Vasoconstriction*

OBJECTIVE: To present the onset of severe pulmonary arterial hypertension (PAH) in a patient with interstitial lung disease (ILD) associated with overlap syndrome. CASE PRESENTATION : A 42-year-old female was diagnosed with overlap syndrome consisting of systemic lupus erythematosus (SLE), systemic sclerosis (SSc) and rheumatoid arthritis (RA). The serologic profi le included positive antinuclear antibody (ANA), anti-dsDNA, anti-RNP, anti-Ro, anti-Scl70, anti-Sm, rheumatoid factor and hypocomplementemia (C3, C4). She had chronic stable ILD for 17 years maintained on hydroxychloroquine (HCQ), prednisone 5 mg/day and indacaterol. The current admission was due to progressive dyspnea and right-sided heart failure over the past month. Chest radiograph showed pulmonary congestion, and 2-dimensional echocardiography (2DE) disclosed severe PAH with systolic pulmonary arterial pressure (SPAP) of 76 mmHg by tricuspid regurgitation (TR) jet, dilated right ventricle (RV) with poor systolic function, moderate pericardial effusion with no signs of tamponade. She received furosemide, beraprost, sildenafi l, and prednisone was increased to 20 mg/ day. Two weeks following discharge, there was complete resolution of symptoms and repeat 2DE showed non-dilated RV with good systolic function, normal SPAP of 21.4 mmHg and minimal pericardial effusion. Prednisone was tapered to 5 mg/day; beraprost, sildenafi l and HCQ were continued. CONCLUSION Overlap syndrome was diagnosed by the combination of clinical features and serology distinctive of SLE, SSc and RA. Her illness, particularly ILD, was adequately controlled over several years, until the recent onset of PAH complicated by right-sided heart failure. The dramatic response to high-dose steroids is more consistent with infl ammatory vasculitis of SLE activity rather than fi brosis typical of SSc.
Pulmonary Arterial Hypertension ; Lung Diseases, Interstitial ; Lupus Erythematosus, Systemic

Heart Defects, Congenital/diagnosis* ; Humans ; Hypertension ; MicroRNAs/genetics* ; Pulmonary Arterial Hypertension ; Pulmonary Artery/diagnostic imaging*

A statistical comparison of pulmonary artery and pulmonary venous wedge pressure has been made by the correlation coefficient method in 24 children with various congenital heart disease. None of them had pulmonary hypertension above the normal range. During the systolic phase, pulmonary arterial pressure was 2.02+/-2.64mmHg greater than pulmonary venous wedge pressure with poor correlation(r=0.57). During the diastolic phase, pulmonary venous wedge pressure was 2.08+/-2.47mmHg greater than pulmonary aetery pressure with poor correlation(r=-.63). Mean pulmonary arterial pressure was 0.79+/-1.02mmHg greater than pulmonary venous wedge pressure with good correlation (r=0.96). Therefore, it is concluded that if pulmonary artery is not entered, a pulmonary vein wedge pressure is a useful indication of pulmonary artery mean pressure in selected cases of congenital heart disease.
Arterial Pressure ; Child ; Heart Defects, Congenital* ; Humans ; Hypertension, Pulmonary ; Pulmonary Artery* ; Pulmonary Veins ; Pulmonary Wedge Pressure* ; Reference Values

As the mitral valve disease becomes long-standing, the patient may develop pulmonary hypertension. It was reported that after surgical correction, the elevated pulmonary vascular resistance(PVR) would fall quickly in association with the fall in left atrial pressure(LAP). This study was performed to evaluate the changes in mean pulmonary artery pressure(PAP) and PVR immediately after mitral valve replacement(MVR). Fifty six patients undergoing mitral valve replacement(MVR) were divided into two groups on the basis of the presence or absence of significant pulmonary hypertension, defined as a resting mean pulmonary arterial pressure greater than 30mmHg. After induction of anesthesia, PAP, PVR, cardiac output(CO) were measured and compared with values in postbypass period. PAP and PVR were significantly decreased(PAP from 39.64+/-1.88 to 29.18+/-1.65 mmHg, P 0.001, PVR from 6.16+/-1.14 to 3.53+/-0.62 units, P<0.05) in Group II(PAP> or = 30mmHg, n=23), whereas not changed in Group I(PAP30 mmHg, n=33)(P<0.05). Persistance of an elevated PVR may cause right ventricular failure and low-output syndrome, so that an attempt to reduce the PVR is needed postoperatiavely. This study demonstrated that the PAP and PVR fall significantly after MVR especially in patients with severe pulmonary hypertension.
Anesthesia ; Arterial Pressure* ; Humans ; Hypertension, Pulmonary ; Mitral Valve* ; Pulmonary Artery ; Vascular Resistance*