Friday morning started off with a special Sunrise Session
, the international pulmonary hypertension challenge. In this session, early career members presented complex cases, and they were followed by an interesting discussion from renowned experts.
In the first case, Julie Wacker, MD
, of University Hospital of Geneva in Switzerland, described a complex case of a 13 year old male who had echo evidence of PH and a PVR of 4.3 wood units on right heart catheterization. CT angiogram showed a large portocaval shunt, and liver biopsy returned with hypoplasia of the intrahepatic portal venous branches. Erika Rosenzweig, MD
, of Columbia University Medical Center in New York, NY USA, led the discussion, commenting on the challenges we face in diagnosis. In particular, she made the point that idiopathic pulmonary arterial hypertension (PAH) is a diagnosis of exclusion. Dr. Rosenzweig reminded us of the necessity of abdominal imaging in the workup of PAH. In this case the patient had portopulmonary hypertension from a congential portocaval shunt, which responded to ligation and vasodilator therapy. We were also reminded by Dr. Rosenzweig that we need to monitor portal pressures with temporary balloon occlusion before ligation of the portocaval shunt.
In the next case, Julien Guihaire, MD, PhD
, of Marie Lannelongue Hospital in Paris, described a complex case of a 57-year-old female who had chronic thromboembolic pulmonary hypertension resulting from antiphospholipid syndrome. The patient underwent pulmonary endarterectomy, but still had a PVR of 9.8 wood units, which persisted despite the addition of riociguat. This was followed by six sessions of balloon pulmonary angioplasty, finally leading to an improvement in the PVR to < 5 wood units. Joanna Pepke-Zaba, MD
, of Royal Papworth Hospital, in Cambridge, UK, then proceeded to discuss the International CTEPH Association’s new Global CTEPH Registry, and the outcomes in the registry, reminding us that with multimodal management we can achieve excellent survival, and improved functional class.
Following these two cases there was a pre-recorded case from Sandeep Sahay, MD
, of Houston Methodist Hospital in Houston, TX USA, about telehealth and remote monitoring in patients with pulmonary arterial hypertension. He discussed a 43-year-old female with HIV-PAH who developed COVID-19 in July 2020, requiring oxygen at discharge. Unfortunately, she had persistent dyspnea, and a worsening of the echocardiogram. Repeat RHC was not allowed because of being persistently COVID-19 positive. The patient was then empirically started on parenteral prostacyclin. The case led to a nice discussion of risk assessment in PAH by Raymond Benza, MD
, of The Ohio State University in Columbus, OH USA, where he highlighted the benefits and pitfalls of using telemedicine to treat and monitor disease progression in patients with PAH. He also introduced the concept of virtual physical exams through a remote device that captures respiratory and heart sounds, new apps which allow for a home six minute walk test, a home ultrasound, and a home BNP kit.
The final case was presented by Nichole Sisserson, MMS, PA-C
, of the Inova Advanced Lung Disease and Transplant Program, USA), and described as case of COVID-19 in a 49-year-old male with pulmonary arterial hypertension. The patient was unable to tolerate PO due to the infection so was switched to IV prostacyclin. Discussant John Granton, MD
, of the University of Toronto, then described the complexities of managing COVID-19 in patients with pulmonary vascular disease including the options for treatment.
– Summary by Nicholas Kolaitis, MD, MAS
Case Study: Is This Really Idiopathic Pulmonary Arterial Hypertension?
Julie Wacker, MD
, University Hospital of Geneva, Geneva, Switzerland
Erika B. Rosenzweig, MD
, Columbia University Medical Center, New York, NY USA
Case Study: BPA for Residual Pulmonary Hypertension Post PEA for CTEPH
Julien Guihaire, MD, PhD
, Marie Lannelongue Hospital, Le Plessis-Robinson, France
Joanna Pepke-Zaba, MD
, Royal Papworth Hospital, Cambridge UK
Case Study: Telehealth and Remote Monitoring in Pulmonary Arterial Hypertension
Sandeep Sahay, MD
, Houston Methodist Hospital, Houston, TX USA
Raymond L. Benza, MD
, The Ohio State University, Columbus, OH USA
Cast Study: Pulmonary Arterial Hypertension and COVID-19
Nichole Sisserson, MMS, PA-C
, Inova Advanced Lung Disease and Transplant Program, Falls Church, VA USA
John Granton, MD
, University of Toronto, Toronto, ON Canada