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1.
Pulm Circ ; 14(2): e12367, 2024 Apr.
Article in English | MEDLINE | ID: mdl-38646413

ABSTRACT

Current predictors of clinical outcomes after pulmonary thromboendarterectomy (PTE) in patients with chronic thromboembolic pulmonary hypertension (CTEPH) are largely limited to preoperative clinical characteristics. N-terminal-pro-brain natriuretic peptide (NT-pro-BNP), a biomarker of right ventricular dysfunction, has not yet been well described as one such predictor. From 2017 to 2021, 816 patients with CTEPH referred to the University of California, San Diego for PTE were reviewed for differences in NT-pro-BNP to predict preoperative characteristics and postoperative outcomes up to 30 days post-PTE. For analysis, NT-pro-BNP was dichotomized to less than/equal to or greater than 1000 pg/mL based on the mean of the study population. Mean NT-pro-BNP was 1095.9 ±1783.4 pg/mL and median was 402.5 pg/mL (interquartile range: 119.5-1410.8). Of the 816 patients included, 250 had NT-pro-BNP > 1000 pg/mL. Those with NT-pro-BNP > 1000 pg/mL were significantly more likely to have worse preoperative functional class (III-IV) and worse preoperative hemodynamics. Patients with NT-pro-BNP > 1000 pg/mL also tended to have more postoperative complications including reperfusion pulmonary edema (22% vs. 5.1%, p < 0.001), airway hemorrhage (8.4% vs. 4.9%, p = 0.075), residual pulmonary hypertension (11.9% vs. 3.1%, p < 0.001), and 30-day mortality (4.8% vs. 1.1%, p = 0.001). Even after adjusting for confounders, patients with NT-pro-BNP > 1000 pg/mL had a 2.48 times higher odds (95% confidence interval: 1.45-4.00) of reaching a combined endpoint that included the above complications. Preoperative NT-pro-BNP > 1000 pg/mL is a strong predictor of more severe preoperative hemodynamics and identifies patients at higher risk for postoperative complications.

2.
Pulm Circ ; 13(4): e12312, 2023 Oct.
Article in English | MEDLINE | ID: mdl-38027455

ABSTRACT

Chronic thromboembolic pulmonary hypertension (CTEPH) is caused by persistent organized thromboembolic obstruction of the pulmonary arteries from incompletely resolved pulmonary embolism. The treatment of choice is pulmonary thromboendarterectomy (PTE) surgery and all patients should be evaluated for operability candidacy. Despite advancements in PTE technique allowing more segmental-subsegmental surgeries, up to a third of patients with CTEPH may still be considered inoperable. Over the past decade, there have been increasing treatment options for these inoperable CTEPH patients. Balloon pulmonary angioplasty (BPA) is a percutaneous-based interventional treatment option for select CTEPH cases. Early BPA experiences were plagued by high complication rates, but further refinements in technique and equipment pioneered by Japan led to the worldwide spread and adoption of BPA. Multiple centers have shown that patients experience significant improvements in hemodynamics, quality of life, exercise capacity, and survival with BPA treatment. There remain many questions on best practices, but BPA has evolved into a pivotal cornerstone of CTEPH treatment.

3.
J Thorac Cardiovasc Surg ; 166(6): 1512-1519.e2, 2023 12.
Article in English | MEDLINE | ID: mdl-37032250

ABSTRACT

OBJECTIVE: Chronic thromboembolic pulmonary hypertension is potentially curable via pulmonary thromboendarterectomy. A minority of patients experience recurrence of their symptoms and are eligible for repeat pulmonary thromboendarterectomy. However, little data exist regarding risk factors and outcomes for this patient population. METHODS: We performed a retrospective review of the University of California San Diego chronic thromboembolic pulmonary hypertension quality improvement database, including all patients who underwent pulmonary thromboendarterectomy from December 2005 to December 2020. Of the 2019 cases performed during this period, 46 were repeat pulmonary thromboendarterectomy procedures. Demographics, preoperative and postoperative hemodynamics, and surgical complications were compared between the repeat pulmonary thromboendarterectomy group and 1008 first pulmonary thromboendarterectomy group. RESULTS: Repeat pulmonary thromboendarterectomy recipients were more likely to be younger, to have an identified hypercoagulable state, and to have higher preoperative right atrial pressure. Etiologies of recurrent disease include incomplete initial endarterectomy, discontinuation of anticoagulation (noncompliance or for medical reasons), and anticoagulation treatment failure. Patients who received repeat pulmonary thromboendarterectomy had significant hemodynamic improvement, but less pronounced compared with patients who received first pulmonary thromboendarterectomy. Repeat pulmonary thromboendarterectomy was associated with an increased risk of postoperative bleeding, reperfusion lung injury, residual pulmonary hypertension, and increased ventilator, intensive care unit, and hospital days. However, hospital mortality was similar between the groups (2.2% vs 1.9%). CONCLUSIONS: This is the largest reported series of repeat pulmonary thromboendarterectomy surgery. Despite an increase in postoperative complications, this study demonstrates that repeat pulmonary thromboendarterectomy surgery can result in significant hemodynamic improvement with acceptable surgical mortality in an experienced center.


Subject(s)
Hypertension, Pulmonary , Pulmonary Embolism , Reperfusion Injury , Humans , Hypertension, Pulmonary/etiology , Hypertension, Pulmonary/surgery , Pulmonary Embolism/surgery , Pulmonary Embolism/complications , Retrospective Studies , Reperfusion Injury/complications , Endarterectomy , Anticoagulants , Chronic Disease
4.
J Heart Lung Transplant ; 42(8): 1112-1119, 2023 08.
Article in English | MEDLINE | ID: mdl-37024310

ABSTRACT

BACKGROUND: Chronic thromboembolic pulmonary hypertension (CTEPH) is primarily managed by pulmonary thromboendarterectomy (PTE). As advanced surgical techniques permit resection at the segmental and subsegmental level, PTE can now be curative for CTEPH mostly involving the distal pulmonary arteries. METHODS: Between January 2017 and June 2021, consecutive patients undergoing PTE were categorized according to the most proximal level of chronic thrombus resection: Level I (main pulmonary artery), Level II (lobar), Level III (segmental) and Level IV (subsegmental). Proximal disease patients (any Level I or II) were compared to distal disease (Level III or IV bilaterally) patients. Demographics, medical history, preoperative pulmonary hemodynamics, and immediate postoperative outcomes were obtained for each group. RESULTS: During the study period, 794 patients underwent PTE, 563 with proximal disease and 231 with distal disease. Patients with distal disease more frequently had a history of an indwelling intravenous device, splenectomy, upper extremity thrombosis or use thyroid replacement and less often had prior lower extremity thrombosis or hypercoagulable state. Despite more use of PAH-targeted medications in the distal disease group (63.2% vs 50.1%, p < 0.001), preoperative hemodynamics were similar. Both patient groups exhibited significant improvements in pulmonary hemodynamics postoperatively with comparable in-hospital mortality rates. Compared to proximal disease, a lower percentage of patients with distal disease showed residual pulmonary hypertension (3.1% vs 6.9%, p = 0.039) and airway hemorrhage (3.0% vs 6.6%, p = 0.047) postoperatively. CONCLUSIONS: Thromboendarterectomy for distal (segmental and subsegmental) CTEPH is technically feasible and may result in favorable pulmonary hemodynamic outcomes, without increased mortality or morbidity.


Subject(s)
Hypertension, Pulmonary , Pulmonary Embolism , Humans , Pulmonary Embolism/complications , Pulmonary Embolism/surgery , Hypertension, Pulmonary/etiology , Hypertension, Pulmonary/surgery , Pulmonary Artery/surgery , Hemodynamics , Endarterectomy/methods , Chronic Disease
5.
Radiographics ; 43(2): e220078, 2023 02.
Article in English | MEDLINE | ID: mdl-36525366

ABSTRACT

Management of chronic thromboembolic pulmonary hypertension (CTEPH) should be determined by a multidisciplinary team, ideally at a specialized CTEPH referral center. Radiologists contribute to this multidisciplinary process by helping to confirm the diagnosis of CTEPH and delineating the extent of disease, both of which help determine a treatment decision. Preoperative assessment of CTEPH usually employs multiple imaging modalities, including ventilation-perfusion (V/Q) scanning, echocardiography, CT pulmonary angiography (CTPA), and right heart catheterization with pulmonary angiography. Accurate diagnosis or exclusion of CTEPH at imaging is imperative, as this remains the only form of pulmonary hypertension that is curative with surgery. Unfortunately, CTEPH is often misdiagnosed at CTPA, which can be due to technical factors, patient-related factors, radiologist-related factors, as well as a host of disease mimics including acute pulmonary embolism, in situ thrombus, vasculitis, pulmonary artery sarcoma, and fibrosing mediastinitis. Although V/Q scanning is thought to be substantially more sensitive for CTEPH compared with CTPA, this is likely due to lack of recognition of CTEPH findings rather than a modality limitation. Preoperative evaluation for pulmonary thromboendarterectomy (PTE) includes assessment of technical operability and surgical risk stratification. While the definitive therapy for CTEPH is PTE, other minimally invasive or noninvasive therapies also lead to clinical improvements including greater survival. Complications of PTE that can be identified at postoperative imaging include infection, reperfusion edema or injury, pulmonary hemorrhage, pericardial effusion or hemopericardium, and rethrombosis. ©RSNA, 2022 Online supplemental material is available for this article.


Subject(s)
Hypertension, Pulmonary , Pulmonary Embolism , Humans , Hypertension, Pulmonary/diagnostic imaging , Hypertension, Pulmonary/etiology , Hypertension, Pulmonary/surgery , Pulmonary Embolism/complications , Pulmonary Embolism/diagnostic imaging , Pulmonary Embolism/surgery , Endarterectomy/adverse effects , Endarterectomy/methods , Angiography/methods , Radiologists , Chronic Disease
6.
Ann Thorac Surg ; 116(1): 121-128, 2023 Jul.
Article in English | MEDLINE | ID: mdl-36368350

ABSTRACT

BACKGROUND: Airway hemorrhage is a known complication of pulmonary thromboendarterectomy (PTE) in patients with chronic thromboembolic pulmonary hypertension. Predisposing factors for postoperative airway hemorrhage have not been well described. The aims of this study were to determine the incidence and outcomes of airway hemorrhage after PTE and to identify potential risk factors. METHODS: This was a retrospective chart review of subjects undergoing PTE between 2015 and 2019. Postoperative airway hemorrhage was defined as significant endobronchial bleeding requiring withholding anticoagulation, bronchial blocker placement, and/or extracorporeal membrane oxygenation (ECMO). RESULTS: Of 877 subjects who underwent PTE, 58 subjects (6.6%) developed postoperative airway hemorrhage. Subjects with hemorrhage were more likely to be women (60% vs 45%, P = .03), to be older (57.8 vs 54.0 years, P = .04), and to have a higher incidence of preoperative hemoptysis (19.0% vs 7.6%, P = .006) compared with control subjects (subjects without airway hemorrhage). Those with hemorrhage had significantly higher preoperative right atrial pressure (P = .002) and pulmonary vascular resistance (P < .001) and a higher incidence of residual pulmonary hypertension (P = .005). Airway hemorrhage management included ECMO with bronchial blocker (n = 2), bronchial blocker without ECMO (n = 26), or withholding anticoagulation alone until bleeding subsided (n = 30). Mortality was significantly higher in those with airway hemorrhage compared with control subjects (13.8% vs 1.2%, P < .001). CONCLUSIONS: The incidence of postoperative airway hemorrhage is low but associated with significant mortality. Older age, female sex, preoperative hemoptysis, and worse preoperative pulmonary hypertension were associated with an increased risk of developing postoperative airway hemorrhage.


Subject(s)
Hypertension, Pulmonary , Pulmonary Embolism , Humans , Female , Male , Pulmonary Embolism/surgery , Pulmonary Embolism/complications , Hypertension, Pulmonary/epidemiology , Hypertension, Pulmonary/etiology , Hypertension, Pulmonary/surgery , Retrospective Studies , Hemoptysis/etiology , Risk Factors , Postoperative Hemorrhage/etiology , Anticoagulants/therapeutic use , Endarterectomy/adverse effects
7.
Pulm Circ ; 12(4): e12148, 2022 Oct.
Article in English | MEDLINE | ID: mdl-36325508

ABSTRACT

Chronic thromboembolic pulmonary hypertension (CTEPH) is the result of incompletely resolved pulmonary emboli (PE) that lead to chronic right heart failure. The two mechanical treatment options are pulmonary thromboendarterectomy (PTE) and balloon pulmonary angioplasty (BPA). There are no formal criteria for BPA patient selection and treatment decisions vary according to a center's experience with BPA and PTE. We performed a retrospective review of consecutive patients treated with PTE and BPA at UCSD from March 2015 to 2021. Clinical and hemodynamic data were collected. Patients were categorized according to the rationale for BPA. One hundred fifty three patients underwent 643 BPA sessions, and 1104 patients underwent PTE. Patients selected for PTE had worse baseline hemodynamics with mean pulmonary artery pressure 41.1 ± 11.7 versus 34.6 ± 11.2 mmHg, p < 0.001. 59% of patients selected for BPA had surgically inaccessible disease, 21% had residual CTEPH after PTE, 10% had a discordance between disease burden and symptoms/hemodynamics, 7% had comorbidities that prevented PTE and 3% refused PTE surgery. 28% of patients who underwent PTE had exclusively level III or IV disease based on surgical specimen. There were no BPA procedure-related mortalities and minor pulmonary vascular complication rates during BPA were 9.2%. The most common reason for BPA selection was surgically inaccessible disease followed by residual CTEPH after PTE. Almost one third of patients who underwent PTE had exclusively distal disease by surgical criteria and might have been directed to BPA at a less experienced surgical center.

8.
Chest ; 162(6): 1360-1372, 2022 Dec.
Article in English | MEDLINE | ID: mdl-35841932

ABSTRACT

The management of pulmonary arterial hypertension (PAH) has become more complex in recent years because of increased pharmacotherapy options and longer patient survival with increasing numbers of comorbidities. As such, more opportunities exist for drug-drug interactions between PAH-targeted medications and medications potentially used to treat comorbid conditions. In this review, we provide an overview of pharmaceutical metabolism by cytochrome P450 and discuss important drug-drug interactions for the 14 Food and Drug Administration-approved medications for PAH in the nitric oxide (NO), endothelin, and prostacyclin pathways. Among the targets in the NO pathway (sildenafil, tadalafil, and riociguat), important interactions with nitrates, protease inhibitors, and other phosphodiesterase inhibitors can cause profound hypotension. In the endothelin pathway, bosentan is associated with more drug interactions via CYP3A4 inhibition; macitentan and ambrisentan have fewer interactions of note. Although the parenteral therapies in the prostacyclin pathway bypass significant liver metabolism and avoid drug interactions, selexipag and oral treprostinil may exhibit interactions with CYP2C8 inhibitors such as gemfibrozil and clopidogrel, which can raise drug levels. Finally, we provide a framework for identifying potential drug-drug interactions and avoiding errors.


Subject(s)
Hypertension, Pulmonary , Pulmonary Arterial Hypertension , Humans , Pulmonary Arterial Hypertension/drug therapy , Familial Primary Pulmonary Hypertension/complications , Bosentan/therapeutic use , Drug Interactions , Antihypertensive Agents/therapeutic use
9.
Pulm Circ ; 12(2): e12066, 2022 Apr.
Article in English | MEDLINE | ID: mdl-35514777

ABSTRACT

Over the past 20 years, despite significant advancements in pulmonary arterial hypertension (PAH) medical therapy, many patients require admission to the hospital and are at risk for in-hospital cardiac arrest (IHCA). Prior data found poor survival in PAH patients after cardiac arrest. The purpose of this study was to explore post-IHCA outcomes in PAH patients receiving advanced medical therapies. This is a single-center retrospective study of PAH patients who underwent cardiopulmonary resuscitation for IHCA between July 2005 and May 2021. Patients were identified through an internal cardiac arrest database. Twenty six patients were included. Half of the cohort had idiopathic PAH, with 54% of patients on combination therapy, 27% on monotherapy, and 19% of patients on no therapy. Mean right atrial pressure, mean pulmonary artery pressure, cardiac index, and pulmonary vascular resistance were 13 ± 6 mmHg, 57 ± 13 mmHg, 2.0 ± 0.7 L/min/m2, and 14.5 ± 7.6 Wood units, respectively. Most common etiology of cardiac arrest was circulatory collapse. Initial arrest rhythm in all but one patient was pulseless electrical activity. Six patients (23%) achieved return of spontaneous circulation (ROSC) and one patient (4%) survived to hospital discharge. Rates of ROSC and survival to discharge after IHCA are poor in patients with PAH. Even patients with mild hemodynamics had low likelihood of survival. In patients who are lung transplant candidates, there should be early consideration of extracorporeal support before cardiac arrest.

10.
Am J Obstet Gynecol MFM ; 3(4): 100358, 2021 07.
Article in English | MEDLINE | ID: mdl-33785463

ABSTRACT

BACKGROUND: Despite the development of advanced therapies for pulmonary arterial hypertension, pregnancy remains contraindicated in these patients owing to high maternal and fetal morbidity and mortality. Limited data exist regarding pregnancy management and outcome in this unique patient population. We describe a series of pregnant patients diagnosed as having pulmonary arterial hypertension before or during pregnancy who delivered at a tertiary center with a comprehensive and established pulmonary vascular disease program. OBJECTIVE: This study aimed to describe a single institution's experience and review the existing literature for pregnancy management and outcomes in patients with pulmonary arterial hypertension. STUDY DESIGN: A review of all patients with pulmonary arterial hypertension who were admitted for delivery between 2005 and 2019 at our institution was performed. All data were extracted from the electronic health record and included patient demographics, pulmonary arterial hypertension subtype, pulmonary arterial hypertension-targeted therapies, and mode of delivery and anesthesia. RESULTS: A total of 7 patients were identified; 5 patients had a prepartum diagnosis of pulmonary arterial hypertension, whereas 2 patients were diagnosed as having pulmonary arterial hypertension during the third trimester. All patients were started on prostacyclins and the majority were on combination pulmonary arterial hypertension-targeted therapy. The maternal mortality rate was 29%. Elective cesarean delivery was performed in more than 70% of cases, whereas 1 patient required an urgent cesarean delivery and 1 patient had a successful vaginal delivery. Most patients had epidural anesthesia. Notably, 2 patients required extracorporeal membrane oxygenation after delivery and both died. There were no cases of neonatal mortality. CONCLUSION: Our cases series and the published literature to date show that pregnancy in pulmonary arterial hypertension remains poorly tolerated despite marked advancements in pulmonary arterial hypertension-targeted therapies and postpartum care. A multidisciplinary team approach remains essential for the management of these patients.


Subject(s)
Hypertension, Pulmonary , Pregnancy Complications, Cardiovascular , Pulmonary Arterial Hypertension , Cesarean Section , Familial Primary Pulmonary Hypertension , Female , Humans , Hypertension, Pulmonary/diagnosis , Infant, Newborn , Pregnancy , Pregnancy Complications, Cardiovascular/diagnosis
11.
Ann Thorac Surg ; 111(5): 1703-1709, 2021 05.
Article in English | MEDLINE | ID: mdl-32896544

ABSTRACT

BACKGROUND: At the University of California, San Diego, routine coronary angiography has generally been performed in men 40 years of age and older and women 45 years of age and older before pulmonary thromboendarterectomy for chronic thromboembolic pulmonary hypertension (CTEPH). The prevalence of significant coronary artery disease (CAD) in this population has not been evaluated, however, and the optimal screening strategy has not been established. This study sought to evaluate whether the current approach may be better optimized on the basis of cardiac risk factors. METHODS: This study included 462 consecutive patients with CTEPH who were undergoing preoperative coronary angiography for pulmonary thromboendarterectomy. Baseline demographic and medical information was recorded. Major cardiac risk factors included: diabetes, hypertension, hyperlipidemia, body mass index 25 kg/m2 or greater, tobacco use, and family history of CAD. Charts were then reviewed for presence of significant CAD and revascularization. RESULTS: Significant CAD was found in 13.4% of patients who underwent routine preoperative coronary angiography; it was present in only 5% of patients younger than 50 years of age, compared with 16% of patients 50 years old and older. No patient younger than 50 years of age without cardiac risk factors was found to have significant CAD. Furthermore, in patients younger than 50 years of age, significant CAD was found only among those with 3 or more major risk factors. CONCLUSIONS: In patients younger than 50 years of age with CTEPH, the prevalence of significant CAD was low. Omitting preoperative coronary angiography in this subset of patients is reasonable when no coronary risk factors are present. Preoperative coronary angiography is warranted in individuals 50 years of age and older, as well as in those younger than 50 years who have significant risk factors for CAD.


Subject(s)
Coronary Angiography , Coronary Artery Disease/complications , Coronary Artery Disease/diagnostic imaging , Endarterectomy , Pulmonary Embolism/complications , Pulmonary Embolism/surgery , Adult , Chronic Disease , Coronary Artery Disease/epidemiology , Female , Humans , Male , Middle Aged , Preoperative Period , Prevalence , Retrospective Studies , Risk Factors
12.
J Am Coll Cardiol ; 76(18): 2155-2169, 2020 11 03.
Article in English | MEDLINE | ID: mdl-33121723

ABSTRACT

Chronic thromboembolic pulmonary hypertension (CTEPH) is the result of pulmonary arterial obstruction by organized thrombotic material stemming from incompletely resolved acute pulmonary embolism. The exact incidence of CTEPH is unknown but appears to approximate 2.3% among survivors of acute pulmonary embolism. Although ventilation/perfusion scintigraphy has been supplanted by computed tomographic pulmonary angiography in the diagnostic approach to acute pulmonary embolism, it has a major role in the evaluation of patients with suspected CTEPH, the presence of mismatched segmental defects being consistent with the diagnosis. Diagnostic confirmation of CTEPH is provided by digital subtraction pulmonary angiography, preferably performed at a center familiar with the procedure and its interpretation. Operability assessment is then undertaken to determine if the patient is a candidate for potentially curative pulmonary endarterectomy surgery. When pulmonary endarterectomy is not an option, pulmonary arterial hypertension-targeted pharmacotherapy and balloon pulmonary angioplasty represent potential therapeutic alternatives.


Subject(s)
Computed Tomography Angiography/methods , Hypertension, Pulmonary/epidemiology , Hypertension, Pulmonary/therapy , Pulmonary Embolism/epidemiology , Pulmonary Embolism/therapy , Anticoagulants/administration & dosage , Chronic Disease , Humans , Hypertension, Pulmonary/diagnostic imaging , Pulmonary Embolism/diagnostic imaging
14.
J Card Surg ; 34(5): 312-317, 2019 May.
Article in English | MEDLINE | ID: mdl-30908728

ABSTRACT

BACKGROUND AND OBJECTIVES: Atrial arrhythmias (AAs) are common after cardiac surgeries including pulmonary thromboendarterectomy (PTE). This study was done to identify patients at highest risk of developing post-PTE AA and their length of stay (LOS). METHODS: We reviewed 521 consecutive patients referred to University of California San Diego (UCSD) for PTE and examined their demographics as well as their baseline pulmonary hemodynamics to determine risk factors for AA. RESULTS: Overall, 24.2% of patients developed an AA after PTE. Patients who developed AA had a significantly longer Intensive Care Unit (ICU) LOS (median: 5 vs 3 days, P < 0.001) and postoperative LOS (median: 14 vs 9 days; P < 0.001). Patients who developed AA were more frequently male (63.2% male, P = 0.003), older (mean age 60.8 vs 50.7 years, P < 0.001), had a prior history of atrial fibrillation (80.2% of those who developed AA) and were more likely to have undergone concomitant Coronary Artery Bypass Graft (12.7% vs 6.6%, P = 0.028). Compared to those who did not develop AA, the cardiopulmonary bypass time was longer among those who developed AA (261.6 vs 253.8 minutes, P = 0.027). In a multivariate logistic regression model, the preoperative variables that predicted AA were age (odds ratio [OR], 1.058 per year, 95% confidence interval [CI]: 1.038-1.078), male sex (OR, 1.68, 95% CI: 1.06-2.64), prior AA (OR, 2.52, 95% CI: 1.23-5.15) and baseline right atrial pressure (OR, 1.039 per mm Hg, 95% CI: 1.000-1.079). While mortality rates were similar, patients who developed AA had more bleeding complications and more postoperative delirium. CONCLUSIONS: AA is common after PTE surgery. The strongest risk factors for AA after PTE included the previous history of AA, age and male sex. Development of AA was associated with longer lengths of stay and more postoperative complications.


Subject(s)
Arrhythmias, Cardiac/epidemiology , Endarterectomy , Postoperative Complications/epidemiology , Pulmonary Embolism/surgery , Adult , Age Factors , Aged , Arrhythmias, Cardiac/etiology , Atrial Fibrillation/complications , Cardiopulmonary Bypass , Female , Humans , Length of Stay , Male , Middle Aged , Operative Time , Postoperative Hemorrhage/epidemiology , Postoperative Hemorrhage/etiology , Risk , Risk Factors , Sex Factors
15.
J Matern Fetal Neonatal Med ; 31(9): 1209-1213, 2018 May.
Article in English | MEDLINE | ID: mdl-28353362

ABSTRACT

BACKGROUND: Epoprostenol, a potent vasodilator, is the treatment of choice for severe pulmonary arterial hypertension (PAH) in pregnancy. However, its inhibition of platelet aggregation increases the risk of coagulation complications with conjunctive use of anti-coagulants for thromboprophylaxis. CASES: Case 1 demonstrates a pregnancy complicated by thrombocytopenia. Case 2 describes a pregnancy with newly diagnosed PAH at 35 weeks who delivered by repeat cesarean delivery complicated by a wound hematoma. Case 3 describes a patient who delivered at 32 weeks. She required extracorporeal membrane oxygenation and a heart-lung transplant. Her care was further complicated by severe thrombocytopenia with postpartum hemorrhage refractory to usual conservative measures. CONCLUSIONS: This case series describes three patients with severe PAH in pregnancy and the range of different complications that arose from anticoagulation in the setting of epoprostenol.


Subject(s)
Anticoagulants/adverse effects , Epoprostenol/adverse effects , Hypertension, Pulmonary/complications , Hypertension, Pulmonary/drug therapy , Platelet Aggregation Inhibitors/adverse effects , Pregnancy Complications, Cardiovascular/drug therapy , Adult , Anticoagulants/therapeutic use , Cesarean Section/adverse effects , Epoprostenol/therapeutic use , Extracorporeal Membrane Oxygenation , Female , Gestational Age , Heart-Lung Transplantation , Hematoma/complications , Humans , Hypertension, Pulmonary/surgery , Platelet Aggregation Inhibitors/therapeutic use , Postpartum Hemorrhage/etiology , Pregnancy , Pregnancy Outcome , Surgical Wound/complications , Thrombocytopenia/chemically induced , Thrombocytopenia/complications
16.
BMC Pulm Med ; 16(1): 82, 2016 05 18.
Article in English | MEDLINE | ID: mdl-27193179

ABSTRACT

BACKGROUND: We present here the case of a patient with pulmonary arterial hypertension and NYHA Class II symptoms who transitioned from PDE-5i therapy to riociguat. No protocol currently exists for transitioning between these PAH medications. CASE PRESENTATION: A 59-year old male with a history of anorexigen use initially presented in 2008 and was felt to have non-operable small vessel disease. His care was transitioned to our center after insurance would not cover high-dose sildenafil in addition to ERA therapy. CONCLUSION: This case demonstrates a safe and successful transition from higher dose PDE-5is to riociguat with no interruption in therapy.


Subject(s)
Familial Primary Pulmonary Hypertension/drug therapy , Phosphodiesterase 5 Inhibitors/therapeutic use , Pyrazoles/therapeutic use , Pyrimidines/therapeutic use , Sildenafil Citrate/therapeutic use , Familial Primary Pulmonary Hypertension/physiopathology , Humans , Insurance Coverage , Male , Middle Aged , Transitional Care
17.
Heart Fail Rev ; 21(3): 309-22, 2016 05.
Article in English | MEDLINE | ID: mdl-26739298

ABSTRACT

Pulmonary hypertension due to chronic thromboembolic disease is potentially curable with pulmonary thromboendarterectomy surgery. As a result, it is important for clinicians to recognize and appropriately diagnose this form of pulmonary hypertension. Advances in this field with changes in surgical technique, the availability of PH-targeted medical therapy for select patient subgroups, and the development of balloon pulmonary angioplasty have broadened therapeutic options for patients. This review will examine what is known about the epidemiology and medical conditions placing patients at risk of developing this disease, will present an approach to evaluation of patients with suspected chronic thromboembolic disease, and will describe the surgical and non-surgical management of this unique patient population.


Subject(s)
Hypertension, Pulmonary/diagnostic imaging , Hypertension, Pulmonary/etiology , Pulmonary Embolism/physiopathology , Venous Thromboembolism/complications , Venous Thromboembolism/physiopathology , Angiography , Angioplasty, Balloon/adverse effects , Chronic Disease , Endarterectomy/adverse effects , Endothelin Receptor Antagonists/therapeutic use , Humans , Hypertension, Pulmonary/therapy , Phosphodiesterase Inhibitors/therapeutic use , Pulmonary Embolism/surgery , Randomized Controlled Trials as Topic , Venous Thromboembolism/surgery
18.
Methodist Debakey Cardiovasc J ; 12(4): 205-212, 2016.
Article in English | MEDLINE | ID: mdl-28289495

ABSTRACT

Chronic thromboembolic pulmonary hypertension (CTEPH) is a potentially curable disease when treated with pulmonary thromboendarterectomy (PTE). However, even at experienced surgical centers, nearly one-third of patients with CTEPH will be deemed inoperable for reasons including distal disease, comorbidities, or out-of-proportion pulmonary hypertension. It is in these patients with inoperable CTEPH that pulmonary hypertension (PH)-targeted medical therapy and balloon pulmonary angioplasty have potential therapeutic value. Previous unblinded cohort trials have assessed PH-targeted medical therapy in various subpopulations of CTEPH patients using epoprostenol, treprostinil, sildenafil, bosentan, and iloprost, each demonstrating measurable pulmonary hemodynamic effects. However, riociguat, a soluble guanylate cyclase stimulator, is the first FDA-approved therapy for inoperable CTEPH to demonstrate both an improvement in functional capabilities (6-minute walk time) as well as significant gains in secondary pulmonary hemodynamic end points in a large placebo-controlled trial. Balloon pulmonary angioplasty is an interventional procedure using telescoping catheters placed in the pulmonary arteries, through which wires and balloons are used to mechanically disrupt chronic clot material and relieve pulmonary vascular obstruction. Contemporary case series from multiple centers worldwide have demonstrated pulmonary hemodynamic improvement with this approach. As a result of these advances, patients with inoperable CTEPH who had few options as recently as 5 years ago now have alternatives with emerging evidence of therapeutic efficacy.


Subject(s)
Angioplasty, Balloon , Antihypertensive Agents/therapeutic use , Arterial Pressure/drug effects , Hypertension, Pulmonary/therapy , Pulmonary Artery/drug effects , Pulmonary Embolism/therapy , Angioplasty, Balloon/adverse effects , Antihypertensive Agents/adverse effects , Chronic Disease , Exercise Tolerance/drug effects , Humans , Hypertension, Pulmonary/diagnosis , Hypertension, Pulmonary/etiology , Hypertension, Pulmonary/physiopathology , Pulmonary Artery/physiopathology , Pulmonary Embolism/complications , Pulmonary Embolism/diagnosis , Pulmonary Embolism/physiopathology , Recovery of Function , Treatment Outcome
19.
Pulm Circ ; 5(2): 313-21, 2015 Jun.
Article in English | MEDLINE | ID: mdl-26064456

ABSTRACT

This study sought to determine the prevalence of coronary artery-pulmonary artery collaterals in patients with chronic thromboembolic pulmonary hypertension (CTEPH) and to correlate their presence with the degree of clot burden. CTEPH is a treatable cause of severe pulmonary hypertension and right heart failure. Bronchopulmonary collateral vessels have been used as a supplementary diagnostic and prognostic tool for this disease. Coronary artery-pulmonary artery collaterals in this population have not been described. The coronary angiograms of 300 consecutive patients with CTEPH evaluated for pulmonary thromboendarterectomy (PTE) between January 1, 2007, and May 1, 2014, were examined. Of these patients, 259 (50% male; mean age, 58.3 ± 10.6 years) had cineangiographic images deemed adequate to definitively assess for the presence of coronary artery-pulmonary artery collaterals and were included in the final analyses. Pulmonary angiogram reports were reviewed for extent of pulmonary artery obstruction. The coronary angiograms of 259 age- and sex-matched control patients were also examined. Among 259 CTEPH patients with definitive imaging, 34 coronary artery-pulmonary artery collaterals were found in 28 patients (10.8%), versus 1 coronary artery-pulmonary artery collateral among control subjects (0.4%; P < 0.001). Compared with CTEPH patients without collaterals, patients with collaterals had a significantly higher prevalence of total occlusion of their right or left main pulmonary artery (P < 0.001) or lobar arteries (P < 0.001). In conclusion, the prevalence of coronary artery-pulmonary artery collaterals in CTEPH patients undergoing coronary angiography for possible PTE is approximately 11%. These vessels are associated with more severe pulmonary artery occlusion.

20.
Ann Thorac Surg ; 98(5): 1776-81, 2014 Nov.
Article in English | MEDLINE | ID: mdl-25240778

ABSTRACT

BACKGROUND: Obesity is a common comorbidity of patients with chronic thromboembolic pulmonary hypertension referred for pulmonary thromboendarterectomy, yet the effect of obesity on pulmonary thromboendarterectomy outcomes has not been well described. METHODS: We conducted a retrospective cohort study in which 476 consecutive operations over a 3.5-year period were examined to determine the effects of obesity on outcomes. Patients were grouped into four categories based on body mass index (BMI): less than 22 kg/m2, 22 to 30 kg/m2, 30 to 40 kg/m2, and more than 40 kg/m2. RESULTS: There were important differences in baseline pulmonary hemodynamics, with obese patients having significantly lower pulmonary vascular resistances than nonobese patients. All patients achieved a significant reduction in pulmonary vascular resistance, although the improvement was greatest in the lower BMI groups. The overall in-hospital mortality was 0.8%, and there were no differences in risk among BMI groups. Among the BMI groups, there were no differences in incidence of postoperative complications, including atrial fibrillation (overall 24.8%), reperfusion lung injury (overall 23.1%), and surgical site infection (overall 4.4%) or in median lengths of stay (including ventilator days, intensive care unit days, and postoperative length of stay). CONCLUSIONS: Pulmonary thromboendarterectomy outcomes have continued to improve, and this surgery can safely be completed in obese patients, previously deemed to be at high risk for poor outcomes.


Subject(s)
Body Mass Index , Endarterectomy/methods , Hypertension, Pulmonary/surgery , Pulmonary Artery/surgery , Pulmonary Embolism/surgery , Thrombectomy/methods , Angiography , California/epidemiology , Cardiac Catheterization , Female , Follow-Up Studies , Humans , Hypertension, Pulmonary/etiology , Hypertension, Pulmonary/mortality , Male , Middle Aged , Pulmonary Embolism/complications , Pulmonary Embolism/mortality , Retrospective Studies , Risk Factors , Survival Rate/trends , Tomography, X-Ray Computed , Treatment Outcome
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