Severe Primary Pulmonary Hypertension Research Articles

Position Statement on the Use of Myocardial Strain in Cardiology Routines by the Brazilian Society of Cardiology's Department Of Cardiovascular Imaging - 2023.

Central Illustration : Position Statement on the Use of Myocardial Strain in Cardiology Routines by the Brazilian Society of Cardiology's Department Of Cardiovascular Imaging - 2023 Proposal for including strain in the integrated diastolic function assessment algorithm, adapted from Nagueh et al.67 Am: mitral A-wave duration; Ap: reverse pulmonary A-wave duration; DD: diastolic dysfunction; LA: left atrium; LASr: LA strain reserve; LVGLS: left ventricular global longitudinal strain; TI: tricuspid insufficiency. Confirm concentric remodeling with LVGLS. In LVEF, mitral E wave deceleration time < 160 ms and pulmonary S-wave < D-wave are also parameters of increased filling pressure. This algorithm does not apply to patients with atrial fibrillation (AF), mitral annulus calcification, > mild mitral valve disease, left bundle branch block, paced rhythm, prosthetic valves, or severe primary pulmonary hypertension.

Inefficacy of Sildenafil treatment of idiopathic pulmonary hypertension

There were studied effects of phosphodiesterase inhibitor Sildenafil on clinical and functional status in young patient with severe primary pulmonary hypertension. Sildenafil was applicated in dose of 50-100 mg daily for 12 weeks, clinical and functional status were estimated initial and after every month of treatment. The retrospective analysis do not detected any positive dynamics in all estimated parameters, this phenomena may be explained by individual patient refractivity on this group of medicine.

A Case of Spontaneously Improved Heritable Pulmonary Arterial Hypertension Diagnosed as Severe Primary Pulmonary Hypertension in Childhood

A Case of Spontaneously Improved Heritable Pulmonary Arterial Hypertension Diagnosed as Severe Primary Pulmonary Hypertension in Childhood

Method selection and perioperative management of termination of pregnancy during the first and second trimester of pregnancy with severe cardiovascular disease

Objective: To summarize and analyze the methods of termination of pregnancy in the first and second trimester of pregnancy with severe cardiovascular disease. Methods: A retrospective analysis of 27 cases of termination of pregnancy in the first and second trimester of pregnancy in Beijing Anzhen Hospital from January 1, 2016 to December 30, 2017. All of these pregnant women were pregnancy complicated with severe cardiovascular disease in grade Ⅴ pregnancy risk. Results: (1) The age of 27 pregnant women was 22-40 years, gestational age was 6-27 weeks; cardiac function grade before induced labor was: 5 cases of grade Ⅱ, 15 cases of grade Ⅲ, 7 cases of grade Ⅳ. The cardiovascular diseases included ventricular septal defect in 9 cases, simple atrial septal defect in 3 cases, patent ductus arteriosus in 2 cases, tetralogy of Fallot in 2 cases, rheumatic valvular disease in 1 case,arrhythmia-paroxysmal atrial velocity in 2 cases, cardiomyopathy in 2 cases, hypertensive cardiopathy in 2 cases, primary pulmonary hypertension in 1 case, tissue disease complicated with pulmonary hypertension 1 case, hyperthyroid heart disease in 1 case, coronary heart disease in 1 case. Among them, 21 cases were complicated with pulmonary hypertension, and 5 cases with Eisenmenger syndrome. (2) Methods of termination of pregnancy: in 27 cases, eight cases of vacuum curettage, and 2 cases of forceps curettage under general anesthesia without intubation; rivanol intraamniotic induction of labor in 1 case; and hysterotomy delivery in 16 cases (11 cases were treated with continuous epidural anesthesia, 1 case was treated with combined spinal and epidural anesthesia, 4 cases were treated with spinal anesthesia) . (3) After termination of pregnancy, one patient with severe primary pulmonary hypertension at 19 weeks of gestation died on the 1st day postoperative. No significant changes in cardiac function were observed in other patients before and after termination of pregnancy, and all of them survived. Conclusions: In patients with severe cardiovascular disease, termination of pregnancy after pregnancy may result in maternal death even at the second trimester of pregnancy.It is strongly recommended that such patients undergo pre-pregnancy assessment and fertility counseling.If visiting doctor after pregnancy, we should organize a multidisciplinary consultation as soon as possible to assess the risk of continuing pregnancy and give medical advice. If the risk of pregnancy is high, we should terminate the pregnancy as early as possible, after making a suitable treatment plan. During termination of pregnancy, temporary or permanent contraception may be taken if the condition permits.

Neuraxial Blockade for Cesarean in a Patient with Severe Primary Pulmonary Hypertension, Tricuspid Insufficient and Right Heart Failure: Case Report

Neuraxial Blockade for Cesarean in a Patient with Severe Primary Pulmonary Hypertension, Tricuspid Insufficient and Right Heart Failure: Case Report

Minimally invasive cardiovascular monitoring in patient with severe pulmonary hypertension for caesarean delivery

Management of severe pulmonary hypertension in pregnancy poses a multitude of problems. This report presents a case of severe primary pulmonary hypertension for elective caesarean section managed successfully under combined spinal epidural anaesthesia with newer minimally invasive cardiac output monitoring.

Gebelikte Ciddi Primer Pulmoner Hipertansiyon

Gebelikte Ciddi Primer Pulmoner Hipertansiyon

Unique Coexistence of Severe Primary Pulmonary Hypertension and Marfanoid Habitus in a Young Male - An Interesting and Rare Case Report

Unique Coexistence of Severe Primary Pulmonary Hypertension and Marfanoid Habitus in a Young Male - An Interesting and Rare Case Report

Intravenous Epoprostenol for Management of Pulmonary Arterial Hypertension during Pregnancy

Background Pulmonary arterial hypertension carries a high risk of mortality in pregnancy. Recent advances in treatment may improve disease course and allow for successful management of the pregnancy.Case Report We present the case of a 20-year-old gravida 1, para 0 with diagnosis of severe primary pulmonary hypertension. The patient was managed with epoprostenol (prostacyclin) infusion via an indwelling catheter, which was initiated at 23 weeks' gestation. The dose was adjusted to the patient's symptoms and a successful vaginal delivery was achieved at 36 weeks' gestation. Although maternal postpartum course was uncomplicated, unexplained neonatal demise occurred at 11 days of life.Conclusion Successful management of pulmonary hypertension in pregnancy can be accomplished with a multidisciplinary approach and intensive therapy. Long-term effects of epoprostenol on fetal or neonatal well-being are unknown.

Primary pulmonary hypertension during pregnancy: A case report

We describe a case of a 25-year-old pregnant woman who presented with severe primary pulmonary hypertension (PPH). Her echocardiogram showed severe right ventricular hypertrophy with dilatation and Moderate right ventricular systolic dysfunction. Right ventricle systolic pressure (RVSP) was estimated to be 125mmHg. She had an elective caesarean section under general anaesthesia at 32weeks of gestation. Pulmonary artery pressures measured by a pulmonary artery catheter before anaesthesia were 102mmHg and pulmonary vascular resistance was 429. Intraoperative nitric oxide was used to reduce pulmonary artery systolic pressure (PASP). After the delivery of a healthy infant, PASP was controlled with nebulized iloprost and silandifil. Five days later she was transferred from intensive care unit after she was started on silandifil 50mg three times daily and a small dose of warfarin.

The use of recombinant tissue-type plasminogen activator in a newborn with an intracardiac thrombus developed during extracorporeal membrane oxygenation

Extracorporeal membrane oxygenation (ECMO) support is often used to support infants and children with hemodynamic or respiratory failure. One of the major obstacles of safely treating a child with ECMO is balancing the risk of hemorrhage with the potential for thrombus development. Managing thrombosis in the setting of ECMO is challenging and has no defined algorithm. The use of recombinant tissue-type plasminogen activator (tPA) for thrombolysis has been previously described in cases where thrombi have developed despite adequate anticoagulation. In such situations, the risk of hemorrhage must be carefully balanced with the benefit of dissolving the clot and reestablishing flow. We present a case of an infant who required ECMO because of severe primary pulmonary hypertension and subsequently developed a right atrial thrombus adjacent to the ECMO cannula. The patient was treated with tPA with immediate improvement but had fatal intracranial hemorrhage almost 3 days after the tPA was administered. In this report, we review the current literature on tPA use during ECMO support and suggest a rational approach.

An Infant with Netherton Syndrome and Persistent Pulmonary Hypertension Requiring Extracorporeal Membrane Oxygenation

Netherton syndrome is a rare genodermatosis characterized by ichthyosiform scaling, hair shaft abnormalities, and atopic features. Affected infants typically have delayed growth and development, immune abnormalities with recurrent infections, and intermittent aminoaciduria. We report a 23-day-old girl who presented with severe primary pulmonary hypertension, exfoliative erythroderma, and trichorrhexis invaginata. Genetic studies confirmed a premature termination mutation R350X in exon 12 of SPINK5. This mutation further supports the genotypic-phenotypic prediction that severe sequela result from premature termination mutations. To our knowledge, this is the first instance of Netherton syndrome associated with primary pulmonary hypertension to be reported. Further postulated is a possible link between excessive desquamation of fetal skin and respiratory failure in a neonate with Netherton syndrome.

Autoimmune thyroid diseases in patients with primary pulmonary hypertension

Abnormal metabolism due to thyroid disorders could cause the additional significant right ventricular (RV) overload in patients with primary pulmonary hypertension (PPH). The purpose of this study was the prospective determination of autoimmune thyroid status in PPH patients. 45 patients (33 women and 12 men, mean age 38 years+/-13) with moderate and severe PPH (mean pulmonary artery pressure 61 mmHg+/-18 mmHg). PPH was diagnosed when other reason of pulmonary hypertension had been excluded especially thromboembolic, venous and hypoxemic. Titer of antibodies to thyroglobulin and thyroid peroxidase were measured. Thyroid gland dysfunction was determined by biochemical measurements of thyrotropin and free thyroxine; thyroid ultrasonographic examination was also performed. The prevalence of thyroid autoantibodies is estimated at 13% in general population. RESULTS. In 14 patients (31%) increased titer of thyroid antibodies was found in our group. Hyperthyroidism was recognized in 2 patients and in 3 cases we diagnosed hypothyroidism with high antibodies level requiring treatment. Ultrasonographic abnormalities were revealed in 21 patients (nodular changes--11 patients, fibrosis--3 patients, chronic inflammation symptoms--7). Ultrasonographic abnormalities were present in all patients with detectable antithyroid antibodies. We haven't confirmed more frequent prevalence of hormonal disorders in patient treated with prostacyclin's analogues. The prevalence of autoimmune thyroid diseases is about 3 times higher in PPH patients than in general population. The treatment of patients with clinically evident hyper- or hypothyroidism is necessary. The monitoring of clinically silent disorders seems to be recommended especially prior to lung transplantation qualification.

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

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. (Korean J Anesthesiol 2008; 54: 473∼7)

Risk of cardiac catheterization under anaesthesia in children with pulmonary hypertension

Children with primary pulmonary hypertension (PHT) are a high-risk group who require assessment by cardiac catheterization under anaesthesia. Complications, including death, have occurred during anaesthesia in these patients, but the true risk has not been quantified. The clinical records of children with PHT undergoing general anaesthesia for pulmonary vascular resistance studies were reviewed retrospectively. Data collected included pre-catheter measures of severity of disease, details of clinical management, and complications occurring within 24 h of the start of anaesthesia. During the past 5 yr, 75 consecutive patients were catheterized and usable records were available in 70. The age range was 0.1-18 yr (mean 7.1). Four children required external cardiac massage [6% (95% confident limits 1-11%)] and one of these died. Of the four, two had an arrhythmia related to the mechanical effects of catheterization, one was hypotensive during anaesthesia and the other had fatal cardiac failure in recovery. All four had severe PHT as judged by echocardiographic estimation of tricuspid regurgitant jet velocity>4 m s-1. Resuscitation or death occurred in 6% of cases. Any associated risk factors could not be determined because the number of complications was too small. Risks may be highest in children with severe idiopathic PHT and symptoms of chest pain, syncope, or dizziness.

Replacing cardiopulmonary bypass with extracorporeal membrane oxygenation in lung transplantation operations☆

Cardiopulmonary bypass (CPB) support is required in some lung transplantation (LTX) operations. CPB support and full-dose heparin increases the risks of bleeding and early graft dysfunction. We report our experiences of replacing CPB with heparin-bonded low-dose heparin extracorporeal membrane oxygenation (ECMO) support in LTX surgery. From 2003 to 2005 forty-seven patients were transplanted. Thirty-seven LTX patients were retrospectively evaluated for this study (10 patients were excluded due to heart-lung-, lung-kidney transplantation, LTX with bypass grafting, and ASD closure or emergency CPB support). Extracorporeal circulation support was necessary in 40% of the 37 LTX patients due to severe primary or secondary pulmonary hypertension (P or SPHTN), right heart dysfunction, or hemodynamic instability. There were seven LTX procedures with CPB and eight implantations with ECMO support. CPB (high-dose heparin) and ECMO support (ACT 160-220 s) was always set up through femoral veno-arterial canulation. All patients had limited access thoracotomies without transsection of the sternum. Normothermia was maintained in all patients. CPB patients: PPH 15%, COPD 15%, IPF with mean PAP>40 mmHg 70%. ECMO patients: PPH 13%, COPD 13%, IPF with severe PAP pressure elevation 74%. In patients undergoing LTX for PPH, the ECMO support was directly extended into the post-operative period. Packed red blood cell (PRBC) transfusion requirements during the operation and the first 24h were 13.25+/-1.6 PRBC units versus 5.1+/-2.8 PRBC units on CBP (p=0.02). Operative time was longer (p=0.11) in the ECMO LTX (451 min+/-76 vs 346+/-140). The increased 90-day mortality rate of the ECMO patients showed a trend toward significance (p=0.056), which was related to infectious complications (3 vs 1 patient). Severe graft ischemia/reperfusion injury occurred in 9% in the CPB versus 13% in the ECMO group. The 1-year survival was significantly reduced in ECMO patients (p=0.004, log-rank test). The advantages of femoral canulation rather than conventional central connections in lung transplantation procedures led to an undisturbed operative field. A significantly higher blood product amount was required in ECMO patients, which might lead to increased infection and mortality rates. CPB, obviously, should remain the standard support technique if extracorporeal circulation is required in lung transplantation surgery.

Pretransplantation Bone Disease in Patients With Primary Pulmonary Hypertension

Osteoporosis is a common condition in patients with end-stage lung disease, but little attention has been given to bone disease in patients with primary pulmonary hypertension (PPH). The purposes of this study were as follows: (1) to determine the prevalence of osteoporosis in patients with severe PPH before lung transplantation, (2) to investigate whether generally accepted risk factors for osteoporosis would play a role in this special group of patients, and (3) to determine whether there is an association between functional parameters and pretransplantion bone mass. A retrospective analysis of 18 consecutive patients with PPH accepted for lung transplantation at the University Hospital of Zurich. Decreased bone mineral density (BMD) [T score <- 1.0] was found in 11 of 18 patients (61%) at the femoral neck (FN) and 13 of 18 patients (72%) at the lumbar spine (LS). We did not find a significant difference of BMD in a gender- or age-specific manner. Body mass index (BMI) correlated significantly with BMD and T score at the FN and WT. Pulmonary vascular resistance (PVR) was notably linked with BMD at the FN. We found a positive association between walking distance in the 12-min walking test and BMD at the FN and WT. Low bone density is a very frequent condition in patients with severe PPH. In contrast to the general population, in our study group neither age nor female gender were significantly associated with lower bone mass. Body weight and BMI were highly coupled with BMD. Among the functional parameters, walking distance and PVR were correlated to BMD at the FN. Considering that patients with PPH may have an improved life expectancy in the future, the early diagnosis, prevention, and treatment of osteoporosis should receive high priority.

Prostacyclin Synthase Promoter Regulation and Familial Pulmonary Arterial Hypertension

Primary pulmonary hypertension (PPH) is a rare disease of unknown etiology characterized by sustained elevations in pulmonary artery pressure. Familial PPH accounts for approximately 6% of cases of PPH and has an autosomal-dominant mode of inheritance. Studies over the last 15 years have identified that both familial and sporadic cases of PPH have an association with mutations in the bone morphogenic protein receptor (BMPR) type II gene. Prostacyclin (PGI2) is currently the standard of care for most patients with severe PPH. Prostacyclin synthase (PGIS) catalyzes the production of PGI2 from prostaglandin H2 and is a critical regulatory step in PGI2 production. Decreased or absent expression of PGIS in the pulmonary microvasculature has been reported in patients with PPH, raising the potential for PGIS as a disease modifying gene. We hypothesize that functional mutations in the PGIS promoter affect transcription regulation of PGI2 production and play a pivotal role in PPH pathogenesis.

Parachute Mitral Valve

A 57-yr-old woman presented to the hospital for further evaluation of recently diagnosed severe primary pulmonary hypertension. Her medical and surgical histories were significant for aortic valvuloplasty in 1961, a St. Jude aortic valve replacement in July 1998, and a subsequent reoperation for the placement of a bioprosthetic aortic valve in September 1998. A permanent pacemaker was placed in 1998 secondary to complete heart block. She experienced increasing shortness of breath, requiring home oxygen for exertion. She had no history of smoking or significant pulmonary history before this diagnosis. Ventilation/perfusion imaging and spiral computed tomography excluded pulmonary embolism. Cardiac catheterization revealed normal coronary arteries, pulmonary artery pressures of 109/42 mm Hg, and a pulmonary capillary wedge pressure of 40 mm Hg. Both transthoracic and transesophageal echocardiography examinations were performed after admission to the hospital. Based on these findings, she underwent a reoperation for placement of a Carpentier-Edwards pericardial mitral valve. The following preoperative and intraoperative transesophageal echocardiography images display the patient's pathology (Figs. 1 and 2) (video clip available at www.anesthesia-analgesia.org).Figure 1.: From this mid-esophageal 4-chamber view the mitral valve appears to be opening adequately at the annular level; however, the mitral valve assumes a “pear-shaped” configuration.Figure 2.: This still-frame image displays a continuous wave Doppler spectral profile representing severe mitral stenosis.The finding of a parachute mitral valve in an adult patient is extremely rare. Most patients are diagnosed in childhood with the pathologic complex known as Shone's Anomaly, which includes a parachute mitral valve and aortic coarctation (68% of patients), atrial septal defect (54% of patients), ventricular septal defect (46% of patients), aortic valve stenosis (32% of patients), subaortic stenosis (20% of patients), and left ventricular hypoplasia (19% of patients) (1). Based on analysis of embryonic specimens, Oosthoek et al. (2) concluded that the development of the anterolateral and posteromedial papillary muscles is disrupted between the 5th and 19th weeks of gestation, thereby forcing the embryonic predecessors of the papillary muscles to condense into a single muscle. This case effectively illustrates how a detailed transesophageal echocardiography examination can aid the physician in clinical decision-making process when presented with a diagnostic dilemma.

Erratum

A 57-yr-old woman presented to the hospital for further evaluation of recently diagnosed severe primary pulmonary hypertension. Her medical and surgical histories were significant for aortic valvuloplasty in 1961, a St. Jude aortic valve replacement in July 1998, and a subsequent reoperation for the placement of a bioprosthetic aortic valve in September 1998. A permanent pacemaker was placed in 1998 secondary to complete heart block. She experienced increasing shortness of breath, requiring home oxygen for exertion. She had no history of smoking or significant pulmonary history before this diagnosis. Ventilation/perfusion imaging and spiral computed tomography excluded pulmonary embolism. Cardiac catheterization revealed normal coronary arteries, pulmonary artery pressures of 109/42 mm Hg, and a pulmonary capillary wedge pressure of 40 mm Hg. Both transthoracic and transesophageal echocardiography examinations were performed after admission to the hospital. Based on these findings, she underwent a reoperation for placement of a Carpentier-Edwards pericardial mitral valve. The following preoperative and intraoperative transesophageal echocardiography images display the patient's pathology (Figs. 1 and 2) (video clip available at www.anesthesia-analgesia.org).Figure 1.: From this mid-esophageal 4-chamber view the mitral valve appears to be opening adequately at the annular level; however, the mitral valve assumes a “pear-shaped” configuration.Figure 2.: This still-frame image displays a continuous wave Doppler spectral profile representing severe mitral stenosis.The finding of a parachute mitral valve in an adult patient is extremely rare. Most patients are diagnosed in childhood with the pathologic complex known as Shone's Anomaly, which includes a parachute mitral valve and aortic coarctation (68% of patients), atrial septal defect (54% of patients), ventricular septal defect (46% of patients), aortic valve stenosis (32% of patients), subaortic stenosis (20% of patients), and left ventricular hypoplasia (19% of patients) (1). Based on analysis of embryonic specimens, Oosthoek et al. (2) concluded that the development of the anterolateral and posteromedial papillary muscles is disrupted between the 5th and 19th weeks of gestation, thereby forcing the embryonic predecessors of the papillary muscles to condense into a single muscle. This case effectively illustrates how a detailed transesophageal echocardiography examination can aid the physician in clinical decision-making process when presented with a diagnostic dilemma.