Congenital absence of the portal vein (CAPV) is a rare malformation in which intestinal and splenic venous flow bypasses the liver and drains directly into the systemic circulation via a congenital portosystemic shunt. We describe two cases of CAPV presenting as pulmonary hypertension that were initially suspected as primary pulmonary hypertension. However, subsequent ultrasonography and CT detected the absence of a portal vein and the presence of a portosystemic shunt. Pulmonary hypertension is a recognized complication of liver disease and portal hypertension. However, these two cases illustrate that CAPV may result in pulmonary hypertension without liver disease or portal hypertension.
Hypertension, Portal ; Hypertension, Pulmonary* ; Liver ; Liver Diseases ; Portal Vein* ; Portasystemic Shunt, Surgical ; Ultrasonography

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.
Adolescent ; Cardiac Catheterization ; Cardiac Catheters ; Chest Pain ; Dizziness ; Dyspnea ; Female ; Headache ; Humans ; Hypertension, Pulmonary* ; Pulmonary Artery ; Pulmonary Wedge Pressure ; Ultrasonography

An 11 year-old girl, whose condition was diagnosed as juvenile-type autosomal recessive polycystic kidney disease (ARPKD) at five years of age, presented with chest pain and dyspnea that had developed suddenly two months previously. Two-dimensional echocardiography, Doppler study and cardiac catheterization confirmed pulmonary hypertension. The underlying mechanism of the diagnosis was not defined. Two and a half months after the onset of symptoms, the patient died of pulmonary hypertensive crisis. Careful regular checks of cardiopulmonary status using two-dimensional echocardiography and Doppler should be considered for the early detection of pulmonary hypertension even in an asymptomatic patient with juvenile-type ARPKD.
Biopsy ; Case Report ; Child ; Echocardiography, Doppler ; Fatal Outcome ; Female ; Human ; Hypertension, Pulmonary/ultrasonography ; Hypertension, Pulmonary/complications* ; Liver Cirrhosis/pathology ; Liver Cirrhosis/complications ; Polycystic Kidney, Autosomal Recessive/ultrasonography ; Polycystic Kidney, Autosomal Recessive/complications*

The 49, XXXXY Klinefelter variant is distinctive; classic findings include mental retardation, hyp ogonadism and radioulnar synostosis. Its incidence is estimated to be 1/80,000 to 1/100,000 male births. We report a case of 49, XXXXY syndrome confirmed by chromosomal study in neonatal period, whose clinical features included a coarse face, short neck, micropenis, and other multiple minor anomalies including simian creases. Echocardiography showed patent ductus arteriosus (PDA), and pulmonary hypertension. A multicystic kidney was found by abdominal ultrasonography.
Ductus Arteriosus, Patent ; Echocardiography ; Humans ; Hypertension, Pulmonary ; Incidence ; Infant, Newborn* ; Intellectual Disability ; Male ; Multicystic Dysplastic Kidney* ; Neck ; Parturition ; Synostosis ; Ultrasonography

A 29-year-old woman in her 8th week of pregnancy was referred to our hospital for swelling in the lower extremities, rapid onset of dyspnea (1 hr) and pre-syncope. Severe right ventricular dysfunction and moderate pulmonary hypertension were detected using 2-dimentional Doppler echocardiography. In addition, left calf vein and proximal thromboses were detected by venous compression ultrasound imaging. After successful thrombolytic treatment, the patient quickly recovered and was discharged from hospital on subcutaneous low-molecular-weight heparin. She delivered a normal, healthy infant at full-term (40 weeks).
Adult ; Dyspnea ; Echocardiography, Doppler ; Female ; Heparin, Low-Molecular-Weight ; Humans ; Hypertension, Pulmonary ; Infant ; Lower Extremity ; Pregnancy* ; Pulmonary Embolism* ; Thrombolytic Therapy ; Thrombosis ; Ultrasonography ; Veins ; Venous Thrombosis* ; Ventricular Dysfunction, Right

This study assessed main pulmonary artery diameter of patients with anthracofibrosis. Patients with anthracofibrosis and CT scans were evaluated after exclusion of patients with co-existing disease. We measured the diameter of the main pulmonary artery (PAD) and ascending aorta (AD) and calculated the pulmonary artery to aorta ratio (APR). The upper reference limit for comparison of PAD was 29 mm. Cut-off values for PAD and APR indicating pulmonary hypertension were 33 mm and 1. We correlated the CT parameters with echocardiographic results. Total 51 patients were included in the analysis. The mean PAD, AD, and APR were 33 mm, 38 mm, and 0.87 respectively. The PAD was larger than the upper reference limit, 29 mm (P<0.001). The PAD was >33 mm in 30 (65%) and the APR was >1 in 9 patients (18%). Of 21 patients with echocardiography, 11 (52%) were found to have pulmonary hypertension. There was no statistical difference in the diagnosis of pulmonary hypertension between echocardiography and CT (P=1.000). In conclusion, main pulmonary artery is dilated in patients with anthracofibrosis more than in the healthy population.
Aged ; Aged, 80 and over ; Aorta, Thoracic/*radiography ; Bronchi/pathology ; Bronchoscopy ; Dilatation ; Female ; Fibrosis ; Humans ; Hypertension, Pulmonary/*diagnosis/ultrasonography ; Male ; Pulmonary Artery/*radiography ; Tomography, X-Ray Computed

Extrinsic compression of the left main coronary artery (LMCA) secondary to pulmonary artery dilatation is a rare syndrome. Most cases of pulmonary artery hypertension but no atherosclerotic risk factors rarely undergo coronary angiography, and hence, diagnoses are seldom made and proper management is often delayed in these patients. We describe a patient that presented with pulmonary hypertension, clinical angina, and extrinsic compression of the LMCA by the pulmonary artery, who was treated successfully by percutaneous coronary intervention. Follow-up coronary angiography showed patent stent in the LMCA in the proximity of the dilated main pulmonary artery. This case reminds us that coronary angiography and percutaneous coronary intervention should be considered in pulmonary hypertension patients presenting with angina or left ventricular dysfunction.
Angina Pectoris/etiology ; Angioplasty, Balloon, Coronary ; Coronary Angiography ; Coronary Stenosis/radiography/therapy ; Coronary Vessels/radiography/*ultrasonography ; Dilatation, Pathologic ; Female ; Humans ; Hypertension, Pulmonary/etiology/radiography ; Middle Aged ; Pulmonary Artery/radiography/*ultrasonography ; Stents ; Tomography, X-Ray Computed ; Ventricular Dysfunction, Left

BACKGROUNDAn echocardiographic right/left ventricular end-diastolic diameter ratio (RV/LV ratio) ≥ 0.9 is an independent predictor of poor prognosis in patients with acute pulmonary embolism. Right ventricular dilation is a common characteristic of both acute pulmonary embolism and idiopathic pulmonary arterial hypertension (IPAH). However, the prognostic value of the RV/LV ratio in patients with IPAH is unknown.

METHODSNinety-five consecutive patients with newly diagnosed IPAH were included, 17 were re-evaluated by echocardiography after 3 - 12 months of targeted therapy. Follow-up data were obtained by telephone interviews and review of the patients' records.

RESULTSHigher RV/LV ratios were associated with greater functional impairment. The RV/LV ratio was positively correlated with pulmonary vascular resistance (r = 0.549, P < 0.001) and plasma N-terminal pro-brain natriuretic peptide level (r = 0.575, P < 0.001), but negatively correlated with cardiac output (r = -0.517, P < 0.001) and mixed venous oxygen saturation (r = -0.599, P < 0.001). Twenty-seven patients died during follow-up period. Sensitivity and specificity of an RV/LV ratio ≥ 0.84 for predicting death were 85.2% and 51.5%, respectively. The RV/LV ratio and body mass index were independent predictors of death by multivariate Cox analysis (P < 0.01). A baseline RV/LV ratio ≥ 0.84 or a further increase in the RV/LV ratio during targeted therapy indicated a poor prognosis (P < 0.01).

CONCLUSIONThe RV/LV ratio helps to assess the severity of IPAH and may serve as an independent predictor of prognosis in patients with IPAH.

Adolescent ; Adult ; Age Factors ; Blood Pressure ; physiology ; Body Mass Index ; Familial Primary Pulmonary Hypertension ; Female ; Heart Ventricles ; diagnostic imaging ; pathology ; Humans ; Hypertension, Pulmonary ; diagnostic imaging ; pathology ; Male ; Prognosis ; Retrospective Studies ; Ultrasonography ; Young Adult

Pulmonary artery sarcoma (PAS) is a rare and fatal disease that often mimics chronic thromboembolic pulmonary hypertension (CTEPH); therefore, diagnosis of PAS is often delayed. Herein, a healthy 74-year-old man was presented with a 4-month history of dyspnea. Chest computed tomography showed wall thickening and stenosis in the main pulmonary artery as well as in both proximal pulmonary arteries. In order to differentiate between unusual CTEPH, vasculitis, and PAS, we performed right heart catheterization and pulmonary angiography. The mean pulmonary arterial pressure was 21 mmHg, and there was severe pulmonary artery stenosis. Thrombi on the pulmonary arterial wall lesions were observed in intravascular ultrasound and optical coherence tomography. Furthermore, the patient had a history of deep vein thrombosis. Therefore, we diagnosed unusual CTEPH. After 6 months of rivaroxaban anticoagulation therapy, a chest X-ray revealed a left lower lobe lung mass, and a positron emission tomography later showed hypermetabolic lesions in the main pulmonary artery wall, in both pulmonary arteries walls, in the lung parenchyma, and in the bones. A biopsy of the right proximal humerus lesion revealed undifferentiated intimal sarcoma. Pulmonary sarcoma is rare, but should be considered when differentially diagnosing main pulmonary artery wall thickening and stenosis. A positron emission tomography may aid in this diagnosis.
Aged ; Angiography ; Arterial Pressure ; Biopsy ; Cardiac Catheterization ; Cardiac Catheters ; Constriction, Pathologic* ; Diagnosis ; Dyspnea ; Fluorodeoxyglucose F18* ; Humans ; Humerus ; Hypertension, Pulmonary ; Lung ; Positron-Emission Tomography ; Positron-Emission Tomography and Computed Tomography* ; Pulmonary Artery* ; Rivaroxaban ; Sarcoma* ; Thorax ; Tomography, Optical Coherence ; Ultrasonography ; Vasculitis ; Venous Thrombosis

Eisenmenger's syndrome is defined as a high pulmonary vascular resistance associated with pulmonary hypertension or high pulmonary pressure close to systemic values with a reverse or bidirectional shunt at aortopulmonary, interventricular or interatrial levels. We report the case of a 42-year-old woman with an emergency operation for ovarian bleeding with Eisenmenger's syndrome secondary to large VSD. She had abdominal pain and vaginal spotting which developed one month earlier. In a preoperative abdominal ultrasonography, there was a fluid collection on the Cul-de-sac. There was no significant cardiorespiratory symptom except peripheral cyanosis. Anesthesia was performed with fentanyl, midazolam and vecuronium in standard monitorings including pulmonary artery pressure monitoring. Bolus and continuous infusions of amrinone were given to decrease right to left shunt. After the administration of amrinone, PaO2, PaO2/FiO2, P(A-a)O2 and P(a/A)O2 were improved and pulmonary arterial pressure was preferentially decreased compared with systemic arterial pressure. There was no significant problem throughout the operation, a right ovarian wedge resection. She was transferred to the intensive care unit in an intubated state postoperatively and discharged one week later without any complications.
Abdominal Pain ; Adult ; Amrinone ; Anesthesia ; Arterial Pressure ; Cyanosis ; Eisenmenger Complex* ; Emergencies ; Female ; Fentanyl ; Hemorrhage* ; Humans ; Hypertension, Pulmonary ; Intensive Care Units ; Metrorrhagia ; Midazolam ; Pulmonary Artery ; Ultrasonography ; Vascular Resistance ; Vecuronium Bromide