John Thenganatt

Pulmonary endarterectomy for chronic thromboembolic pulmonary hypertension: the Toronto experience.

BACKGROUND Pulmonary endarterectomy (PEA) is the treatment of choice for patients with chronic thromboembolic pulmonary hypertension (PH). However, this surgery remains performed in few experienced centres only. The goal of the study is to review our overall experience since the implementation of our program in August 2005. METHODS Review all patients referred to our program between August 2005 and July 2011. RESULTS Among 84 consecutive patients referred to our program, 52 patients underwent elective PEA and 6 emergency PEA. After PEA, 74% patients were extubated within 2 days, 71% were discharged from the intensive care unit within 4 days and 64% were discharged from hospital within 15 days. One patient undergoing elective surgery and 2 patients undergoing emergency surgery died within 30 days of surgery for an operative mortality of 1.9% after elective pulmonary endarterectomy and an overall operative mortality of 5.2%, when the 6 emergency operative cases were included. The total pulmonary vascular resistance decreased from 965±445 to 383±162 dynes per second per cm(-5) and was associated with significant improvement in World Health Organization/New York Heart Association (WHO/NYHA) functional class, 6 minutes walk distance, echocardiographic findings, and brain natriuretic peptide level at 6 months after PEA. After a median follow-up of 23 months (1-65 months), 3 patients had to be started on targeted PH therapy for deterioration of their (WHO/NYHA) functional class. CONCLUSIONS Elective PEA can be performed with limited risk, and results in excellent early and long-term outcome. All patients diagnosed with chronic thromboembolic PH should be referred for consideration of PEA in a specialized centre.

Pulmonary endarterectomy in severe chronic thromboembolic pulmonary hypertension

BACKGROUND The outcome of patients with chronic thromboembolic pulmonary hypertension (CTEPH) undergoing pulmonary endarterectomy (PEA) after urgent hospitalization for decompensated right heart failure (DRHF) remains unclear. METHODS Among 120 consecutive patients undergoing PEA, 16 (13%) presented with a history of urgent hospitalization for DRHF (severe RHF group). This group was compared with the remaining 104 patients presenting with total peripheral vascular resistance (TPR) < 1,200 dynes · sec · cm(-5) (n = 78, control group) and >1,200 dynes · sec · cm(-5) (n = 26, high TPR group). RESULTS DRHF occurred predominantly in patients with TPR > 1,200 dynes · sec · cm(-5) (prevalence of 32% vs 5% in patients with TPR < 1,200 dynes · sec · cm(-5), p < 0.0001). The overall in-hospital mortality after PEA was 4% (n = 5). All deaths occurred in patients with TPR > 1,200 dynes · sec · cm(-5) and DRHF. The proportion of patients with residual PH immediately after surgery was higher in the high TPR group (50%) and severe RHF group (56%) compared with the control group (29%; p = 0.04). In multivariate analysis, risk factors for residual PH after surgery were TPR > 1,200 dynes · sec · cm(-5), Jamieson disease Type III-IV, and female gender. A history of DRHF by itself was not a risk factor for residual PH after PEA. The overall 5-year survival was 87%. After a median follow-up of 20 months, the proportion of patients receiving medical therapy for residual PH was higher in patients with post-operative mean pulmonary artery pressure ≥35 mm Hg (61% vs 9%, p = 0.0007). CONCLUSIONS DRHF occurs more frequently in patients with TPR > 1,200 dynes · sec · cm(-5), increasing the operative risk in these patients. The outcome of patients with high TPR in the absence of DRHF is excellent. However, patients with residual mean pulmonary artery pressure ≥35 mm Hg frequently receive pulmonary hypertension therapy after PEA.

Feasibility of blood conservation strategies in pulmonary endarterectomy for chronic thromboembolic pulmonary hypertension

Blood transfusion requirements were reviewed for a consecutive series of 25 patients undergoing elective pulmonary endarterectomy (PEA) between August 2005 and March 2009 in our institution. Patients were divided into two groups based on the implementation of a conservative blood transfusion algorithm that combined antifibrinolytic therapy, intraoperative blood sequestration, blood salvage and lack of correction of coagulation parameters in the absence of ongoing bleeding. Despite similar perioperative coagulation profiles in the two groups, the introduction of a conservative blood transfusion algorithm was associated with a significant increase in the number of patients receiving no homologous blood products. Of 16 patients who underwent surgery after the introduction of the algorithm, nine (56%) required no homologous blood products and five (31%) required one or two units of homologous red blood cells only. The international normalized ratio normalized within six to 12 hours after discontinuation of cardiopulmonary bypass without transfusion of fresh frozen plasma or platelets in 13 of the 16 patients. In conclusion, a conservative blood transfusion strategy allows PEA to be safely performed with no or minimal blood product transfusions in a majority of patients despite deep hypothermic circulatory arrest.

Dramatic response to sirolimus in lymphangioleiomyomatosis.

Depatrment of Respirology, University of Toronto, Toronto General Hospital, Toronto, Ontario Correspondence: Dr Dmitry Rozenberg, Depatrment of Respirology, Toronto General Hospital, 9N 965 – 585 University Avenue, Toronto, Ontario M5G 2N2. Telephone 416-340-4591, fax 416-340-3109, e-mail dmitry.rozenberg@uhn.ca A 41-year-old woman presented with a one-year history of progressive dyspnea, early satiety and abdominal cramping. A clinical diagnosis of lymphangioleiomyomatosis (LAM) was made based on a confirmed right-sided chylous effusion (milky, exudative and triglyceride level of 52.6 mmol/L) and characteristic cystic changes on a highresolution computed tomography scan of the chest (Figure 1). A large left-sided retroperitoneal lymphangioleiomyoma supported the diagnosis (Figure 2), with no renal angiomyolipoma or clinical evidence of tuberous sclerosis. She was started on sirolimus therapy, which resolved her dyspnea and abdominal symptoms within six months. Her forced expiratory volume in 1 s (FEV1) (1.6 L) and FVC (2.1 L) had markedly improved during this time interval to 2.6 L and 4.2 L, respectively. Her chest x-ray demonstrated almost complete resolution of the right pleural effusion (Figure 3) and the lymphangioleiomyoma had also decreased in size (Figure 4). The patient remains clinically stable on sirolimus with few side effects and her FEV1 and FVC on therapy at nine months increased to 3.3 L (96%) and 4.8 L (118%), respectively. The present case is a dramatic example of a chylous pleural effusion and massive intra-abdominal lymphangioleiomyoma responding to sirolimus therapy. images in respiratory meDicine

Role of extracorporeal life support after pulmonary endarterectomy: a single-centre experience

OBJECTIVES Extracorporeal life support (ECLS) for rescue after pulmonary endarterectomy (PEA) has become a viable option. This study aims to present a single-centre experience looking at the indications and outcome of ECLS after PEA. METHODS Retrospective analysis of all patients undergoing PEA from January 2008 to January 2015 in our institution. RESULTS Among 144 consecutive patients undergoing PEA for chronic thromboembolic pulmonary hypertension, 6 (4%) received ECLS postoperatively for right ventricular (RV) failure (n = 3), severe hypoxaemia (n = 2) and haemorrhagic pulmonary oedema (n = 1). ECLS configuration was central veno-arterial (cVA) in 3 patients, peripheral VA (pVA) in 1 and veno-venous (VV) in 2. One patient with cVA was switched to VV after 5 days. Overall ECLS duration ranged between 3 and 39 (median 5) days. ECLS patients had higher preoperative total pulmonary vascular resistance (TPR) compared with non-ECLS patients (1477 ± 671 vs 954 ± 462 Dynes.s.cm(-5), P = 0.009) and more frequently required hospital admission for RV failure before surgery (50 vs 9%, P = 0.02). The overall in-hospital mortality rate for all patients was 2% (3/144), including one ECLS patient on pVA. The remaining 5 ECLS patients (83%) were discharged from the hospital and are alive after a median follow-up of 11 (range 6-27) months. Two ECLS patients (40%) are on therapy for residual PH compared with 13 (10%) in the non-ECLS patients (P = 0.09). CONCLUSIONS ECLS is a safe and important rescue option after PEA. The use of ECLS may expand eligibility for PEA by allowing sicker patients to undergo surgery.

Exhaled Nitric Oxide in Systemic Sclerosis Lung Disease

Background. Exhaled nitric oxide (eNO) is a potential biomarker to distinguish systemic sclerosis (SSc) associated pulmonary arterial hypertension (PAH) and interstitial lung disease (ILD). We evaluated the discriminative validity, feasibility, methods of eNO measurement, and magnitude of differences across lung diseases, disease-subsets (SSc, systemic lupus erythematosus), and healthy-controls. Methods. Consecutive subjects in the UHN Pulmonary Hypertension Programme were recruited. Exhaled nitric oxide was measured at 50 mL/s intervals using chemiluminescent detection. Alveolar and conducting airway NO were partitioned using a two-compartment model of axial diffusion (CMAD) and the trumpet model of axial diffusion (TMAD). Results. Sixty subjects were evaluated. Using the CMAD model, control subjects had lower median (IQR) alveolar NO than all PAH subjects (2.0 (1.5, 2.5) versus 3.14 ppb (2.3, 4.0), p = 0.008). SSc-ILD had significantly lower median conducting airway NO compared to controls (1009.5 versus 1342.1 ml⁎ppb/s, p = 0.04). SSc-PAH had increased median (IQR) alveolar NO compared to controls (3.3 (3.0, 5.7) versus 2.0 ppb (1.5, 2.5), p = 0.01). SSc-PAH conducting airway NO inversely correlated with DLCO (r −0.88 (95% CI −0.99, −0.26)). Conclusion. We have demonstrated feasibility, identified that CMAD modeling is preferred in SSc, and reported the magnitude of differences across cases and controls. Our data supports discriminative validity of eNO in SSc lung disease.

A rare cause of hemoptysis

Angiosarcomas are rare, malignant, endothelial-cell tumors of vascular origin that can arise at any body site. They frequently metastasize to the lung, heralded by dyspnea, hemoptysis, chest pain, pneumothoraces, and diffuse pulmonary hemorrhage. However, in most cases lung metastases are discovered after the diagnosis of a primary angiosarcoma has already been established. Very rarely will an undiagnosed metastatic angiosarcoma present as diffuse pulmonary hemorrhage. We describe the case of a 59-year-old male who presented to hospital with dyspnea and hemoptysis. CT chest revealed rapidly progressing nodular changes and broncho-alveolar lavage returns were progressively bloody. Open lung wedge biopsy ultimately revealed metastatic angiosarcoma and extensive pulmonary hemorrhage. Our case highlights the key clinical, radiological, and pathological features of this rare malignancy that frequently metastasizes to the lung and reminds clinicians to consider it as a cause of hemoptysis and pulmonary hemorrhage.

A case of pulmonary arterial hypertension associated with adult hemophagocytic lymphohistiocytosis

Hemophagocytic lymphohistiocytosis (HLH) is an aggressive, life-threatening syndrome of excessive immune activation. Presentation is most common among the pediatric population, and cases in adults are rare. The number of nonhematologic presentations described in relation to HLH has been growing. We present a case involving a woman who developed HLH after autologous stem cell transplantation for mantle cell lymphoma. Months later, she received a diagnosis of pulmonary arterial hypertension (PAH) while undergoing treatment for her HLH. To our knowledge, PAH associated with adult HLH has only been described in the literature once before. PAH may now be a potential differential diagnosis for patients with HLH who present with respiratory symptoms.

Connect the dots: a rare case of spontaneous haemopneumothorax

A 19-year-old woman presented to her local emergency department with acute chest pain and shortness of breath. She denied any cough, haemoptysis, fever or leg swelling. Her past medical history was significant for premature birth at 28 weeks gestational age. She required surgical correction of a right clubfoot as an infant. In addition, she had intestinal malrotation complicated by caecal volvulus and perforation treated with laparotomy at age 13, and an ovarian cystectomy performed at age 17. There was no family history of pneumothorax or respiratory disease. She was a non-smoker on no medications. On examination, she was tachycardic, tachypnoeic and had an oxygen saturation in the low 80s. Her respiratory exam demonstrated decreased air entry to bilateral lung bases. A …