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Non-Cirrhotic Intrahepatic Portal Hypertension Induced Pulmonary Arterial Hypertension: Is it More Prevalent than we Think?
The 23rd Annual Scientific Meeting HFSA Introduction: In CHF patients, medical therapy optimization (MTO) remains the mainstay of management. However, attaining MTO can be challenging in patients with advanced CHF due to their symptomatic and hemodynamic intolerance. We present a case of a patient with advanced CHF and rectal cancer in whom slowly cautious MTO succeeded in improving cardiac function and candidacy for cancer chemotherapy and surgery. Case: A 57-year-old male with a medical history of advanced nonischemic CHF (EF 10-15%), a-fib, and rectal well-differentiated adenocarcinoma stage IIA (diagnosed 1 month ago) with GI bleeding and defecation pain presented with dyspnea on minimal exertion and orthopnea. He also had dizziness with standing due to symptomatic hypotension. SBP was between 100-115 mmHg. MRI pelvis showed T3 rectal mass with no enlargement of lymph nodes. Further imaging showed no distant metastases. Due to his advanced CHF, the patient was considered at high risk for surgery or chemotherapy. Rectal palliative radiation therapy was performed. He agreed on hospice palliative care for symptomatic relief. From Cardiology stand of point, the decision was made to continue MTO aiming for myocardial recovery. Radical adjustment of regimen and initiation of medications at miniscule doses eventually led to myocardial recovery with EF of 51% (figure 1). Patient’s SOB and dizziness improved. Cancer progressed to stage IIIb. Due to his improvement, the patient became at lower cardiac risk for other cancer therapy options. As a result, Oncology plan changed to proceed with FOLFOX (5-FU/ Leucovorin/ Oxaliplatin) chemotherapy and surgery. Discussion: In our patient, advanced therapy with left ventricular assist device was not possible given his risk of worsening GI bleeding on anticoagulation. Heart transplantation was not an option given the risk of cancer progression on immunosuppressive therapy. In the context of the patient’s condition and comorbidities, the best option left was to proceed with cautious MTO with clinical and hemodynamic monitoring. MTO resulted in clinical and cardiac function improvement. This gave the patient the window to become a candidate for other cancer therapy options that were not available for him before. Conclusion: Medical therapy optimization in all patients with CHF should always be sought. Despite being challenging under certain circumstances, as seen in our patient, slow and cautious MTO can eventually result in significant improvement in clinical status, cardiac function, and candidacy for other therapy options for other comorbidities.
Figure 1. Demonstrates Patient’s Medical Therapy Optimization over Time. Newly Introduced Medications are in Green, Increased Doses are in Blue, and Cancer Diagnosis is in Red. Abbreviations: BID = Twice Daily, BP = Blood Pressure, EF = Ejection Fraction, HR = Heart Rate, LA = Left Atrial, LVIDd = Left Ventricular Internal Dimension at End Diastole, MUGA = Multigated Acquisition, NYHA = New York Heart Association, QD = Daily, RV = Right Ventricular, TTE = Transesophageal Echocardiogram.
320 Non-Cirrhotic Intrahepatic Portal Hypertension Induced Pulmonary Arterial Hypertension: Is it More Prevalent than we Think? Sarine Beukian1, Ewelina Wojtaszek1, Lori A. Reyes1, Ramyashree Tummala2, Matthew I. Tomey1,3, Barry A. Love1,3, Johanna P. Contreras1,3, Maria G. Trivieri1,3; 1 Mount Sinai Hospital, New York, NY; 2Mount Sinai Beth Israel, New York, NY; 3 Icahn School of Medicine, New York, NY Introduction: Porto-Pulmonary Hypertension (PoPH) belongs to WHO group I, Pulmonary Arterial Hypertension (PAH). It has a prevalence of 0.5-5% and is potentially curable with liver transplant. Non-cirrhotic intrahepatic portal hypertension (NCIPH) is a rare cause of PoPH that shares clinical features of advanced right heart failure. Based on review of the literature and Pulmonary Hypertension (PH) registries, most studies report the prevalence of patients with the liver cirrhosis with little to no data on the incidence of PoPH amongst the non-cirrhotic patient population. In this report, we describe the two cases of “missed” NCIPH and PoPH who were diagnosed in our institution within the span of six months. Case Reports: The first patient was a 36 year old African American female who carried an initial diagnosis of SLE and ITP. She was on combination therapy with prostacyclin and endothelin receptor antagonist for the treatment of PAH. Her recurrent epistaxis from thrombocytopenia was refractory to therapy with prednisone, Rituximab, Romiplostim requiring multiple PRBC transfusions. Abdominal imaging revealed splenomegaly while liver biopsy demonstrated Nodular Regenerative Hyperplasia (NRH) without cirrhosis. The second patient, a 44 year old Chinese female, was initially diagnosed with idiopathic PAH. She was also identified to have ITP and showed no significant response to dexamethasone. On hospital admission, both patients presented with large volume ascites, splenomegaly and dependent edema. While in the ICU, they underwent RHC with assessment of Hepatic Venous Pressure Gradient (HVPG) demonstrating severely
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elevated pressures at 14 mmHg (Normally 5 mmHg) (Table 1). Liver biopsy was performed during the same procedure without complications. The pathology was consistent with NCIPH. Summary: Our report illustrates the importance of considering the diagnosis of NCIPH in patients with PH and refractory ITP, particularly in the setting of splenomegaly and lack of overt cirrhosis. Due to an estimated 50% of patients with NCIPH that go on to develop portal hypertension, it is important to consider this diagnosis when evaluating patients with PH as the treatment strategy differs from idiopathic and connective tissue disease-associated PAH. Given the limited risk of HVPG measurement as part of the evaluation of patients with PAH, our single center experience would suggest screening for NCIPH in select patients and embracing this etiology in the differential. Table 1.
Patient A Patient B
m Hepatic V Wedge
Hepatic Vein P
HVPG
28 28
14 14
14 14
321 Rock at the LVAD Core: Inadvertent Calcified Apical Aneurysm during LVAD Implantation Mohamad Khaled Soufi1, Loui A. Rejjal2, Kaitlyn S. Schaefer3, Tawnya R. Jackson3, Patrick T. Roughneen4, Ghannam A. Al-Dossari4, Jaime A. Hernandez-Montfort1; 1 Advanced Heart Failure and Transplant Cardiology, University of Texas Medical Branch, Galveston, TX; 2Department of Cardiology, University of Texas Medical Branch, Galveston, TX; 3Texas Transplant Center, University of Texas Medical Branch, Galveston, TX; 4Division of Cardiothoracic Surgery, Department of Surgery, University of Texas Medical Branch, Galveston, TX Introduction: Patients with ischemic cardiomyopathy (ICMP) and advanced CHF are at risk to develop calcified ventricular aneurysms as part of their ischemic remodeling over time. These aneurysms can significantly interfere with left ventricular assist device (LVAD) implantation. We present a case of a patient who had a calcified apical aneurysm that was preoperatively missed and inadvertently discovered during his LVAD surgery. Case: A 70 year-old male with a medical history of ICMP (EF 510%, NYHA II, Stage D) status post ICD placement on the heart transplant waiting list, CAD status post CABG (11 years ago), and a-fib presented for an elective LVAD implantation as a bridge to transplant due to worsening in his kidney function and declining in his myocardial oxygen consumption. The patient had CXR and TTE before his planned surgery as part of preoperative work up. During the operation, a calcified apical aneurysm was discovered and resected (figure 1). Afterward, the LVAD swing ring was fixed to the LV apical ventriculotomy followed by the LVAD inflow graft insertion. Thereafter, the operation was completed as planned. The patient recovered and was eventually discharged home. Discussion: Pre-LVAD identification of any calcified myocardial aneurysm is important especially when it is apical as the LVAD inflow graft cannot be sutured over calcified myocardial tissue. In our patient, CXR and TTE failed to identify his calcified apical aneurysm before surgery. Fortunately, the size of his calcified aneurysm was not very big and the surgeon was able to appropriately fix the LVAD swing ring on LV apical ventriculotomy after resecting the aneurysm. Retrospectively, our patient’s long history of ICMP, very low cardiac function, and dilated LV (end-diastolic diameter of 7.4 cm) were all unnoticed hints toward possible calcified aneurysmal formation that would have been
Figure 1. Demonstrates the Patient’s Calcified Apical Aneurysm (yellow Arrows) During its Resection.
Figure 1. Demonstrates Patient’s Medical Therapy Optimization over Time. Newly Introduced Medications are in Green, Increased Doses are in Blue, and Cancer Diagnosis is in Red. Abbreviations: BID = Twice Daily, BP = Blood Pressure, EF = Ejection Fraction, HR = Heart Rate, LA = Left Atrial, LVIDd = Left Ventricular Internal Dimension at End Diastole, MUGA = Multigated Acquisition, NYHA = New York Heart Association, QD = Daily, RV = Right Ventricular, TTE = Transesophageal Echocardiogram.
320 Non-Cirrhotic Intrahepatic Portal Hypertension Induced Pulmonary Arterial Hypertension: Is it More Prevalent than we Think? Sarine Beukian1, Ewelina Wojtaszek1, Lori A. Reyes1, Ramyashree Tummala2, Matthew I. Tomey1,3, Barry A. Love1,3, Johanna P. Contreras1,3, Maria G. Trivieri1,3; 1 Mount Sinai Hospital, New York, NY; 2Mount Sinai Beth Israel, New York, NY; 3 Icahn School of Medicine, New York, NY Introduction: Porto-Pulmonary Hypertension (PoPH) belongs to WHO group I, Pulmonary Arterial Hypertension (PAH). It has a prevalence of 0.5-5% and is potentially curable with liver transplant. Non-cirrhotic intrahepatic portal hypertension (NCIPH) is a rare cause of PoPH that shares clinical features of advanced right heart failure. Based on review of the literature and Pulmonary Hypertension (PH) registries, most studies report the prevalence of patients with the liver cirrhosis with little to no data on the incidence of PoPH amongst the non-cirrhotic patient population. In this report, we describe the two cases of “missed” NCIPH and PoPH who were diagnosed in our institution within the span of six months. Case Reports: The first patient was a 36 year old African American female who carried an initial diagnosis of SLE and ITP. She was on combination therapy with prostacyclin and endothelin receptor antagonist for the treatment of PAH. Her recurrent epistaxis from thrombocytopenia was refractory to therapy with prednisone, Rituximab, Romiplostim requiring multiple PRBC transfusions. Abdominal imaging revealed splenomegaly while liver biopsy demonstrated Nodular Regenerative Hyperplasia (NRH) without cirrhosis. The second patient, a 44 year old Chinese female, was initially diagnosed with idiopathic PAH. She was also identified to have ITP and showed no significant response to dexamethasone. On hospital admission, both patients presented with large volume ascites, splenomegaly and dependent edema. While in the ICU, they underwent RHC with assessment of Hepatic Venous Pressure Gradient (HVPG) demonstrating severely
S119
elevated pressures at 14 mmHg (Normally 5 mmHg) (Table 1). Liver biopsy was performed during the same procedure without complications. The pathology was consistent with NCIPH. Summary: Our report illustrates the importance of considering the diagnosis of NCIPH in patients with PH and refractory ITP, particularly in the setting of splenomegaly and lack of overt cirrhosis. Due to an estimated 50% of patients with NCIPH that go on to develop portal hypertension, it is important to consider this diagnosis when evaluating patients with PH as the treatment strategy differs from idiopathic and connective tissue disease-associated PAH. Given the limited risk of HVPG measurement as part of the evaluation of patients with PAH, our single center experience would suggest screening for NCIPH in select patients and embracing this etiology in the differential. Table 1.
Patient A Patient B
m Hepatic V Wedge
Hepatic Vein P
HVPG
28 28
14 14
14 14
321 Rock at the LVAD Core: Inadvertent Calcified Apical Aneurysm during LVAD Implantation Mohamad Khaled Soufi1, Loui A. Rejjal2, Kaitlyn S. Schaefer3, Tawnya R. Jackson3, Patrick T. Roughneen4, Ghannam A. Al-Dossari4, Jaime A. Hernandez-Montfort1; 1 Advanced Heart Failure and Transplant Cardiology, University of Texas Medical Branch, Galveston, TX; 2Department of Cardiology, University of Texas Medical Branch, Galveston, TX; 3Texas Transplant Center, University of Texas Medical Branch, Galveston, TX; 4Division of Cardiothoracic Surgery, Department of Surgery, University of Texas Medical Branch, Galveston, TX Introduction: Patients with ischemic cardiomyopathy (ICMP) and advanced CHF are at risk to develop calcified ventricular aneurysms as part of their ischemic remodeling over time. These aneurysms can significantly interfere with left ventricular assist device (LVAD) implantation. We present a case of a patient who had a calcified apical aneurysm that was preoperatively missed and inadvertently discovered during his LVAD surgery. Case: A 70 year-old male with a medical history of ICMP (EF 510%, NYHA II, Stage D) status post ICD placement on the heart transplant waiting list, CAD status post CABG (11 years ago), and a-fib presented for an elective LVAD implantation as a bridge to transplant due to worsening in his kidney function and declining in his myocardial oxygen consumption. The patient had CXR and TTE before his planned surgery as part of preoperative work up. During the operation, a calcified apical aneurysm was discovered and resected (figure 1). Afterward, the LVAD swing ring was fixed to the LV apical ventriculotomy followed by the LVAD inflow graft insertion. Thereafter, the operation was completed as planned. The patient recovered and was eventually discharged home. Discussion: Pre-LVAD identification of any calcified myocardial aneurysm is important especially when it is apical as the LVAD inflow graft cannot be sutured over calcified myocardial tissue. In our patient, CXR and TTE failed to identify his calcified apical aneurysm before surgery. Fortunately, the size of his calcified aneurysm was not very big and the surgeon was able to appropriately fix the LVAD swing ring on LV apical ventriculotomy after resecting the aneurysm. Retrospectively, our patient’s long history of ICMP, very low cardiac function, and dilated LV (end-diastolic diameter of 7.4 cm) were all unnoticed hints toward possible calcified aneurysmal formation that would have been
Figure 1. Demonstrates the Patient’s Calcified Apical Aneurysm (yellow Arrows) During its Resection.