Relationship between Right Ventricular Longitudinal Strain, Invasive Hemodynamics, and Functional Assessment in Pulmonary Arterial Hypertension

BACKGROUND AND OBJECTIVES: Right ventricular longitudinal strain (RVLS) is a new parameter of RV function. We evaluated the relationship of RVLS by speckle-tracking echocardiography with functional and invasive parameters in pulmonary arterial hypertension (PAH) patients. SUBJECTS AND METHODS: Thirty four patients with World Health Organization group 1 PAH (29 females, mean age 45+/-13 years old). RVLS were analyzed with velocity vector imaging. RESULTS: Patients with advanced symptoms {New York Heart Association (NYHA) functional class III/IV} had impaired RVLS in global RV (RVLS(global), -17+/-5 vs. -12+/-3%, p<0.01) and RV free wall (RVLS(FW), -19+/-5 vs. -14+/-4%, p<0.01 to NYHA class I/II). Baseline RVLS(global) and RVLS(FW) showed significant correlation with 6-minute walking distance (r=-0.54 and r=-0.57, p<0.01 respectively) and logarithmic transformation of brain natriuretic peptide concentration (r=0.65 and r=0.65, p<0.01, respectively). These revealed significant correlations with cardiac index (r=-0.50 and r=-0.47, p<0.01, respectively) and pulmonary vascular resistance (PVR, r=0.45 and r=0.45, p=0.01, respectively). During a median follow-up of 33 months, 25 patients (74%) had follow-up examinations. Mean pulmonary arterial pressure (mPAP, 54+/-13 to 46+/-16 mmHg, p=0.03) and PVR (11+/-5 to 6+/-2 wood units, p<0.01) were significantly decreased with pulmonary vasodilator treatment. RVLS(global) (-12+/-5 to -16+/-5%, p<0.01) and RVLS(FW) (-14+/-5 to -18+/-5%, p<0.01) were significantly improved. The decrease of mPAP was significantly correlated with improvement of RVLS(global) (r=0.45, p<0.01) and RVLS(FW) (r=0.43, p<0.01). The PVR change demonstrated significant correlation with improvement of RVLS(global) (r=0.40, p<0.01). CONCLUSION: RVLS correlates with functional and invasive hemodynamic parameters in PAH patients. Decrease of mPAP and PVR as a result of treatment was associated with improvement of RVLS.

Primary pulmonary hypertension in pregnancy: Vaginal delivery with epidural labour analgesia.

Primary pulmonary hypertension is a very rare, progressive, incurable disease, the only curable option being heart lung transplant. When pregnancy is associated with pulmonary hypertension due to any cause, it carries very poor prognosis with mortality rate ranging from 30-50%. More risk is involved during labour & peripartum period, as labour pain with hypercarbia, hypoxia, acidosis, increases sympathomimetic responses and pulmonary vascular resistance which could be fatal to parturient. Epidural labour analgesia with painless vaginal delivery attenuates these responses & improves survival rate. It also helps in accommodating the auto transfused blood in postpartum period due to controlled vasodilatation & so avoiding right ventricular failure. Case report: We report a case of a primigravida patient with primary pulmonary hypertension who was advised therapeutic abortion, but she continued with pregnancy and underwent vaginal delivery with epidural analgesia & was continuously haemodynamically monitored non-invasively during labour & postpartum period.

Pulmonary Hypertension: A Review.

Pulmonary hypertension is a relatively common disorder that leads to right heart failure if untreated. Symptoms and signs of pulmonary hypertensionare often subtle and nonspecific. As a result a significant delay between the onset of symptoms and the diagnosis of pulmonary hypertension is common. Recently improved understanding of the pathophysiology of pulmonary hypertension leads to various treatment options that enable us to treat this disorder more efficiently.

Hipertensión pulmonar en paciente VIH positiva / Pulmonary arterial hypertension in a HIV positive patient

Se presenta el caso clínico de una paciente de 30 años, con diagnóstico de VIH + desde el año 2000, en terapia antirretroviral desde el año 2004. Desde el año 2007 con disnea de esfuerzo progresiva, que en el último año se hace severa, asociada a astenia, fatigabilidad y edema vespertino de extremidades inferiores. El ecocardiograma detecta un aumento de la presión de arteria pulmonar, lo que se confirma a través de un cateterismo cardíaco derecho. Se descarta tromboembolismo pulmonar y se concluye como una HAP secundaria a VIH. La hipertensión arterial pulmonar (HAP) es una enfermedad de baja frecuencia y se puede asociar a infección por virus de la inmunodeficiencia humana (VIH), presentando una prevalencia 6 a 12 veces mayor en relación a individuos sin infección por VIH. La HAP es independiente del recuento de linfocitos T CD4 y en su patogenia participan proteínas virales, como la glicoproteína 120 y las proteínas Nef y Tat, que estimulan una cascada inflamatoria sistémica, induciendo angiogénesis en los vasos pulmonares. El estudio hemodinámico a través de cateterismo derecho es el método de elección para la confirmación diagnóstica.

We report the case of a patient 30 years, diagnosed with human immunodeficiency virus (HIV) since 2000, on antiretroviral therapy since 2004. Since 2007 with progressive dyspnea, which in the past year becomes severe, associated with asthenia, fatigue and edema of lower limbs evening. Echocardiography detects an increase in pulmonary artery pressure, which is confirmed by a right heart catheterization. Pulmonary embolism is ruled out and concludes as a pulmonary arterial hypertension (PAH) secondary to HIV. Pulmonary arterial hypertension is a disease of low frequency and can be associated with infection by HIV, with prevalence 6 to 12 times greater relative to individuals without HIV infection. PAH is independent of CD4 T lymphocyte count and viral proteins involved pathogenesis, such as glycoprotein 120 and Nef and Tat proteins, which stimulate a systemic inflammatory cascade, inducing angiogenesis in the pulmonary vessels. The hemodynamic study through right heart catheteri-zation is the method of choice for diagnostic confirmation.

Anesthetic Management in a Patient with Severe Primary Pulmonary Hypertension with Right Ventricular Dysfunction: A case report / 대한마취과학회지

Primary pulmonary hypertension is characterized by progressive increase in pulmonary vascular resistance leading to right ventricular (RV) failure and death.The desirable goal of primary pulmonary hypertension is preserving coronary perfusion of RV while decreasing pulmonary arterial pressure with selective pulmonary vasodilators.We report a case in which anesthetic management was successfully performed in a 67 years old man, who had experienced ventricular tachycardia at the previous anesthesia induction, with severe pulmonary hypertension and right ventricular dysfunction.

Left Main Stem Bronchus Compression Occurred in Primary Pulmonary Hypertension Patient: A case report / 대한마취과학회지

Eleven year-old boy was scheduled for right encephalo-duro arterial synangiosis (EDAS). He received left EDAS 4 months ago, and there was no problem during the perioperative period. Mild cardiomegaly was suspected in his preoperative chest PA. After intubaton, we couldn't hear the breathing sound of left chest, and the saturation went down to 95%. There was large opaque hemithorax on emergency chest AP, and on the fiberoptic bronchoscopic examination, left main stem bronchus (LMSB) was observed slit-like appearance. Echocardiography revealed severely dilated right atrium and ventricle. In his chest CT, the LMSB was nearly obliterated. The patient was diagnosed as primary pulmonary hypertension, and received full sedation, controlled ventilation, and NO gas. However, the blood pressure of this patient was not maintained in spite of using high-dose inotropics. He expired two days later and this case warns that suspected mild cardiomegaly might hide more ominous sign of primary pulmonary hypertension.

A case of pregnancy combined with maternal primary pulmonary hypertension / 대한산부인과학회지

Primary pulmonary hypertension is a rare disorder and rarely present in pregnant woman, but it make high risk of maternal morbidity and mortality. When a woman with subclinical primary pulmonary hypertension is pregnant, physiologic increase in maternal plasma volume aggravates pulmonary pressure cause cardiac arrest and maternal death. We diagnosed primary pulmonary hypertension in a pregnant woman who didn't present any symptoms before pregnancy but had dyspnea from 2nd trimester of gestation. She expired due to sudden aggravation of pulmonary overloading. We reports this case with brief review of literature.

A Case of Primary Pulmonary Hypertension in Pregnancy Presented as Massive Hemoptysis / 결핵및호흡기질환

Primary pulmonary hypertension (PPH) is a rare, progressive and incurable disease, which is characterized by an increase in the pulmonary artery pressure without a demonstrable cause. The most common presenting symptom is dyspnea on exertion, with other symptoms comprising of chest pain, syncope and hemoptysis. The diagnosis is one of exclusion of any of the known causes of pulmonary hypertension. When associated with pregnancy, the maternal mortality ranges from 30 to 50%. Because pregnancy and labor are very serious problems for patients with PPH, the available evidence suggests that pregnancy when afflicted with PPH should be avoided. In account the case of a 33-year old patient, reporting with massive hemoptysis, and diagnosed with PPH during her twenty seventh week of gestation, is presented. She was treated with conservative management, including oxygen and a vasodilator, and underwent a pregnancy termination. However, due to aggravation of right heart failure, she presented with severe systemic hypotension and hypoxemia, and eventually died. This case is reported, with brief review of the literature.

Clinical analysis of primary pulmonary hypertension in children / 重庆医科大学学报

Objective:To analyze the clinical data of primary pulmonary hypertension in children and improve its clinical diagnosis and treatment.Methods:The clinical data of 6 patients with primary pulmonary hypertension were collected and analyzed.Clinical diagnosis was made by ways of electrocardiography,ultrasound,and X-ray.All of them were received catheterization examination and angiography.Results:Heart failures were found in all patients with PPH.Signs of pulmonary hypertension were found by accessory examination.No heart malformation was found by catheterization examination.Conclusion:There are all kinds of clinical manifestations of PPH.The definite diagnosis of PPH will rely on catheterization examination.

A Case of Primary Pulmonary Hypertension Diagnosed in Puerperium

Primary pulmonary hypertension is a condition characterized by sustained elevations of pulmonary artery pressure without demonstrable cause. It is incurable and progressive clinical entity. The incidence has been estimated at approximately 1 to 2 cases per million people in the general population. The maternal mortality of primary pulonary hypertension in pregnancy was said to be 30% and the first month after delivery represents the period of highest risk. We experienced a 27-year-old women with primary pulmonary hypertension diagnosed in postpartum period. She was transferred from the department of obstetrics to internal medicine due to dyspnea on the first day of postpartum. She was diagnosed by perfusion lung scan, chest CT and confirmed by echocardiography. This diagnosis were supported by findings of pulmonary angiogram and cardiac catheterization. Initially she was treated with diuretics, aspirin and anticoagulant therapy, and as a result of the treatment, her symptoms improved. However after 8months has elapsed, exertional dyspnea was worsening. Thus she was treated with calcium channel blocker, aspirin and diuretics and was followed at 1 month interval on the outpatient clinics.

Survival and Prognostic Factors in Patients with Primary Pulmonary Hypertension

OBJECTIVES: Primary pulmonary hypertension (PPH) that affects predominantly young and productive people is a progressive fatal disease of unknown cause. The objectives of this study were to characterize mortality in patients with PPH and to investigate the factors associated with their survival. METHODS: Thirteen patients with PPH were enrolled between 1988 and 1996 and followed-up through July 1999. Measurements at diagnosis included hemodynamic and pulmonary function variables in addition to information on demographic data and medical history. RESULTS: 1) The mean age of the patients with PPH enrolled into the study was 36.1+/-9.3 years with female predominance. 2) The estimated median survival was 3.4+/-0.6 years. 3) Decreased cardiac index was the only significant predictor of mortality (Cox proportional hazards model). CONCLUSION: Patients with PPH have a poor survival expectancy. In this limited study with a small number of patients, mortality is largely associated with decreased cardiac index.

A Case of Primary Pulmonary Hypertension with the Congenital Absence of Inferior Vena Cava

Primary pulmonary hypertension (PPH) is one of rare and fatal vascular disorder. The exact mechanisms for the increased pulmonary vascular resistance and pressure in PPH are unknown. The disease is advanced pro-gressively and patients naturally die within three years after the initial diagnosis. PPH is characterized with mean pulmonary artery pressure over 25-30 mmHg and intractable hypertension despite any intensive therapeutic maneuvers. Associated congenital anomaly is very rare. We report a 19 year-old female patient who diagnosed as PPH 10 years ago and suffered from severe pulmonary hypertension (100 mmHg of pulmonary arterial pressure) with the congenital absence of inferior vena cava. Her venous blood from lower body was drained through azygous vein into superior vena cava.

An Autopsied Case of Primary Pulmonary Hypertension

A twenty four-year-old female patient had suffered progressive dyspnea for 6 years until death. She denied any symptoms suggestive of connective tissue disease, or deep vein thrombosis. She suffered an episode of pontine infarct in 1995. Four years after diagnosis of primary pulmonary hypertension, she died of sudden death during hospitalization. Gross features of pulmonary arteries at autopsy were as follows: left main pulmonary artery showed dilation of the lumen and thickening of the wall, and right main pulmonary artery was markedly dilated and contained fresh thrombus. Hematoxylin and eosin-stained sections of lung tissue showed plexiform lesions of pulmonary arteries, complete luminal obliteration of pulmonary arterioles and dilated lesion of pulmonary arterioles, and capillaries. This patient represents a typical case with a primary pulmonary arteriopathy with plexiform lesions with thrombotic lesion, demonstrating the importance of thrombosis in situ in the pathogenesis of primary pulmonary hypertension. To our knowledge, this is the first autopsy report on the primary pulmonary hypertension in Korea.

A Case of Primary Pulmonary Hypertension Associated with Pregnancy

Primary pulmonary hypertension is a rar, incurable, and progressive clinical entity. When associated with pregnancy, the prognosis of primary pulmonary hypertension is worsened with maternal mortality rates of at least 50%. The patient was a 29-year-old mother in her 2nd pregnancy, with previous uncomplicated gestation, 5 years ago. She had been well until the 28th week of present gestation when she was admitted because of increasing dyspnea on exertion. She was a housewife with no remarkable family and past histories. We have experienced a patient with primary pulmonary hypertension in pregnancy. She was treated with an oral calcium-channel blocker and low-molecular-weight heparin and was delivered vaginally with good maternal and fetal outcome. So we report a case of primary pulmonary hypertension associated with pregnancy and review literature.

A Scanning Electron Microscopic Study on Microvascular Changes in the Monocrotaline-induced Rat Lung by Corrosion Casting Method

To investigate the microvascular changes in primary pulmonary hypertension, the lungs of 24 Sprague-Dawley rats were treated by an intraperitoneal injection of 2% monocrotaline(MCT) solution and then examined with scanning electron microscopy(SEM) after microvascular corrosion casting. Histologic examination revealed significant medial thickening in the small to medium-sized pulmonary arteries. Scanning electron microscopic findings of the normal lungs showed two kinds of microvascular structures. One showed a well-fortned three-dimensional basket structure of uniform flat-tubular alveolar capillaries, which were connected to each other in a T or Y shape or at right angles. The other revealed a two-dimensional reticular sheet of round tubular branches mainly in the bronchial artery-supplying regions. The MCT-treated groups(remodelling) showed apparent changes in both kinds of microvasculatures in comparison to the normal group but the more prominent change was found in Lbe bronchial artery microvasculature showing the dense thick encasement around large pulmonary arteries. Alveolar microvasculature of the pulmonary artery revealed individually enlarged angular appearance, with generally deformed alveolar architecture. Quantitatively, the significant enlargement of diameter and intercapillary distance appeared in both microvasculatures of MCT-induced rat lungs, but the density was increased only in the bronchial artery microvasculature. In conclusion, our three-dimensional microvascular study of the MCT-treated rat lungs demonstrates a new morphologic finding of vascular remodeling in primary puhnonary hypertension, which is thought to play an important vascular role in the pathogenesis in addition to interstitial fibrosis.

Acute Myocardial Infarction in 14-Year-Old Male of Primary Pulmonary Hypertension with Left Ventricular Hypertrophy : A Case Report

Primary pulmonary hypertension is a rare and progressive disease with poor prognosis. Despite much previous studies, there is neither clear explanation in the etiology and the pathogenesis nor confirmative treatment modalities. Its main cause of death is due to the right ventricular failure but the left ventricular function is relatively well preserved. The chest pain mimickig the angina pectoris is common and it is mostly due to the right ventricular ischemia or distension of pulmonary artery, but left ventricular ischemia or infarction is very rarely seen. We experienced a case of primary pulmonary hyperetension with left ventricular hypertrophy that complicated to acute myocardial infarction in 14-year-old male.

A case of primary pulmonary hypertension

We have experienced a 14 year old female patient who had suffered from headache, dizziness, exertional dyspnea and chest pain during 6 months. She was diagnosed as primary pulmonary hypertension by ultrasonogram and cardiac cathererization. On the cardiac catheterization, there was elevated pulmonary artery pressure and normal pulmonary wedge pressure. We report this case with related literature review.

Primary Pulmonary Hypertension: An Analysis of 11 Cases and a Review of Literlature

Primary pulmonary hypertension (PPH) is a rare disease of the pulmonary vasculature which largely affects young women and usually runs a rapidly fatal course. Therapeutic trial has continued to reduce the vascular obstruction, lowering the pulmonary vascular pressure and resistance. However, because the etiology is still unknown, therapy is empiric and most patients are clinically fragile, many physicians refrain from intervening. We analyzed clinical and hemodynamic features of 11 PPH patients, confirmed by echocardiogram and cardiac catheterization, and reviewed the literature. The results were followings: 1) Among 11 patients, 5 were male and 6 female, male to female ratio was 1:1.2. Age ranged from 16 and to 52 years, the mean age was 30.4 years. 2) One patient was associated with scleroderma, the other one case was combined with systemic lupus erythematosus and in two patients, including the latter case, the ANA titer was more than 1:80. 3) Clinical features comprised dyspnea in all, chest pain in 54.5%, palpitation in 45.5%, cyanosis and orthostatic dyspnea in 27.3%, and syncope in 18.2%, respectively. 4) Pulmonic second heart sound was accentuated in all patients. Ejection systolic murmur was heard at pulmonic area in 72.7% and diastolic murmur in 45.5%. Pretibial pitting edema in 36.4%, hepatomegaly and superficial neck vein distension in 27.3% were noted. 5) Right ventricular hypertrophy, right axis deviation, right atrial enlargement and right bundle branch block were common EKG findings. In the chest roentgenogram, prominent pulmonary conus was noted in all and cardiomegaly 9 cases. 6) Echocardiogram showed right ventricular dilatation in all, and the ratio of the thickeness of the interventricular septum and left ventricular posterior wall was 0.88-1.50. Midsystolic notching was demonstrated in 6 patients and abnormal interventricular wall motion in 5. 7) hemodynamic data revealed normal pulmonary wedge pressure or left atriae pressure (ranged 4-14: mean; 8.5+/-3.9mmHg). Meanwhile, pulmonary artery pressure highly elevated: systolic pressure ranged between 60-180 (mean; 107.5+/-30mmHg, diastolic pressure 25-90 (mean; 47.9+/-19.6)mmHg and mean pulmonary artery pressure 40-120(mean; 66.5+/-24.7)mmHg. Pulmonary vascular resistance also elevated between 465-3167 (mean; 1209+/-797) and Rp/Rs was elevated in all. Arterial oxygen desaturation was noted 3 patients.