Browsing by Author "Johansen, Michael"

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    Chronic Thromboembolic Pulmonary Hypertension Successfully Treated in a Six Year Old with Asthma
    (authorea, 2020) Serrano, Ryan; Montgomery, Gregory; Rao, Rohit; Kivett, Tisha; Johansen, Michael; Pediatrics, School of Medicine
    Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare but serious, sequala of acute pulmonary embolism. Symptoms can be subtle and non-specific and the prognosis is poor if severe pulmonary hypertension (PH) and right ventricular dysfunction are present. While PH-targeted therapies are often used, there is only one FDA approved therapy, and only for disease that is deemed inoperable. The greatest chance for potential cure and long-term survival is surgical pulmonary endarterectomy. We report a 6-year-old male with a history of asthma and two unprovoked deep venous thromboses who presented with syncope. Chest x-ray showed cardiomegaly and an echocardiogram showed severe PH with severely decreased right ventricular (RV) function. Ventilation-perfusion scan showed mismatched perfusion defects involving the right lower lobe, and CT of the chest showed right lower lobe subsegmental pulmonary thrombus, suspicious for chronic thromboembolism. Given his clinical presentation and the severity of his right ventricular dysfunction, he was started on ambrisentan, sildenafil and subcutaneous treprostinil. Bilateral pulmonary endarterectomy was performed with resection of level 2 to 3 disease and he was successfully weaned off all PH therapy. Four months post-op, he is clinically asymptomatic and his echo shows normal RV function without PH. In conclusion, CTEPH is a rare but likely underdiagnosed disease process in pediatrics. Clinicians should have a high index of suspicion for at risk patients with unexplained dyspnea. Even if right ventricular dysfunction and severe PH are present, surgical pulmonary endarterectomy can be performed successfully in young children.
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    Combination therapy for severe portopulmonary hypertension in a child allows for liver transplantation
    (Wiley, 2019-08) Serrano, Ryan M.; Subbarao, Girish C.; Mangus, Richard S.; Montgomery, Greg; Johansen, Michael; Pediatrics, School of Medicine
    Severe PPHTN is a contraindication to liver transplantation and predicts an abysmal 5‐year outcome. It is defined as a resting mPAP >45 mm Hg with a mean pulmonary artery wedge pressure of <15 mm Hg and pulmonary vascular resistance of >3 wood units in the setting of portal hypertension. There have been limited reports of successful treatment of PPHTN leading to successful liver transplantation in adults, and one reported use of monotherapy as a bridge to successful liver transplant in pediatrics. To our knowledge, we describe the first use of combination therapy as a successful bridge to liver transplantation in a pediatric patient with severe PPHTN. This report adds to the paucity of data in pediatrics on the use of pulmonary vasodilator therapy in patients with severe PPHTN as a bridge to successful liver transplantation. Early diagnosis in order to mitigate or avoid the development of irreversible pulmonary vasculopathy that would preclude candidacy for liver transplantation is crucial, but our report demonstrates that combination therapy can be administered safely, quickly, and may allow for successful liver transplantation in patients with severe PPHTN.
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    Early ascertainment of genetic diagnoses clarifies impact on medium-term survival following neonatal congenital heart surgery
    (American Society for Clinical Investigation, 2024-07-30) Landis, Benjamin J.; Helm, Benjamin M.; Durbin, Matthew D.; Helvaty, Lindsey R.; Herrmann, Jeremy L.; Johansen, Michael; Geddes, Gabrielle C.; Ware, Stephanie M.; Pediatrics, School of Medicine
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    Learning to Crawl: Determining the Role of Genetic Abnormalities on Postoperative Outcomes in Congenital Heart Disease
    (AHA, 2022-10) Landis, Benjamin J.; Helm, Benjamin M.; Herrmann, Jeremy L.; Hoover, Madeline C.; Durbin, Matthew D.; Elmore, Lindsey R.; Huang, Manyan; Johansen, Michael; Li, Ming; Przybylowski, Leon F.; Geddes, Gabrielle C.; Ware, Stephanie M.; Pediatrics, School of Medicine
    Background Our cardiac center established a systematic approach for inpatient cardiovascular genetics evaluations of infants with congenital heart disease, including routine chromosomal microarray (CMA) testing. This provides a new opportunity to investigate correlation between genetic abnormalities and postoperative course. Methods and Results Infants who underwent congenital heart disease surgery as neonates (aged ≤28 days) from 2015 to 2020 were identified. Cases with trisomy 21 or 18 were excluded. Diagnostic genetic results or CMA with variant of uncertain significance were considered abnormal. We compared postoperative outcomes following initial congenital heart disease surgery in patients found to have genetic abnormality to those who had negative CMA. Among 355 eligible patients, genetics consultations or CMA were completed in 88%. A genetic abnormality was identified in 73 patients (21%), whereas 221 had negative CMA results. Genetic abnormality was associated with prematurity, extracardiac anomaly, and lower weight at surgery. Operative mortality rate was 9.6% in patients with a genetic abnormality versus 4.1% in patients without an identified genetic abnormality (P=0.080). Mortality was similar when genetic evaluations were diagnostic (9.3%) or identified a variant of uncertain significance on CMA (10.0%). Among 14 patients with 22q11.2 deletion, the 2 mortality cases had additional CMA findings. In patients without extracardiac anomaly, genetic abnormality was independently associated with increased mortality (P=0.019). CMA abnormality was not associated with postoperative length of hospitalization, extracorporeal membrane oxygenation, or >7 days to initial extubation. Conclusions Routine genetic evaluations and CMA may help to stratify mortality risk in severe congenital heart disease with syndromic or nonsyndromic presentations.
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    Preventative practices and effects of the COVID-19 pandemic on caregivers of children with pediatric pulmonary hypertension
    (BMC, 2022-12) Nelson, Erik J.; Cook, Ella; Pierce, Megan; Nelson, Samara; Bangerter Seelos, Ashley; Stickle, Heather; Brown, Rebecca; Johansen, Michael; Pediatrics, School of Medicine
    Background Pulmonary hypertension (PH) is a serious and life-threatening disease characterized by elevated mean arterial pressure and pulmonary vascular resistance. COVID-19 may exacerbate PH, as evidenced by higher mortality rates among those with PH. The objective of this study was to understand the unique burdens that the COVID-19 pandemic has placed upon families of children living with PH. Methods Participants were recruited online through the “Families of children with pulmonary hypertension” Facebook group and asked to complete a survey about their experiences during the COVID-19 pandemic. Results A total of 139 parents/caregivers of children living with PH completed the online survey. Almost all (85.6%) of parents/caregivers had received the COVID-19 vaccine, though only 59.7% reported a willingness to vaccinate their child with PH against COVID-19. Over 75% of parents/caregivers felt that they practiced preventative measures (e.g., wearing a facemask, social distancing, and avoiding gatherings) more than those in the community where they live. They also reported several hardships related to caring for their child with PH during the pandemic such as financial duress, loss of work, and affording treatment costs. Conclusions These findings indicate that parents/caregivers of children at higher risk for COVID-19 complications may be more willing to act on clinical recommendations themselves as proxy for protecting those at high risk. The economic, emotional and social impacts of COVID-19 are significantly greater for high-risk individuals.
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    Quantifying side effects and caregiver burdens of pediatric pulmonary hypertension therapies
    (BMC, 2023-01) Nelson, Erik J.; Cook, Ella; Nelson, Samara; Brown, Rebecca; Pierce, Megan; Bangerter Seelos, Ashley; Stickle, Heather; Johansen, Michael; Pediatrics, School of Medicine
    Background and objectives Pulmonary hypertension (PH) is a rare, but serious disease among children. However, PH has been primarily evaluated among adults. Consequently, treatment therapies have not been fully evaluated among pediatric populations and are used in an ‘off label’ manner. The purpose of this study was to estimate the side effect profiles of the most commonly prescribed pediatric PH therapies and to understand the burdens placed upon families caring for children living with PH. Methods Participants were recruited online through the “Families of children with pulmonary hypertension” Facebook group and asked to complete a survey about PH treatments. Results A total of 139 parents of a child living with PH completed the survey. Almost all children used ≥ 1 medication to treat PH, with 52% using ≥ 3 medications. The highest average number of side effects was reported by users of Treprostinil, Selexipag and type-5 phosphodiesterase (PDE5) inhibitors. The most common side effects were skin flushing, headache, nasal congestion, joint/muscle pain, and nausea. In terms of accessing care, 81% travel ≥ 20 miles and 68% travel for ≥ 60 min to receive care. Conclusions We found an array of treatment combinations employed to mitigate symptoms of PH in children, with a wide range of side effects. We also found a large, unseen economic, emotional, and time burden of caring for a child living with PH. Further research is warranted to understand the clinical implications of these side effects to move towards labeled usage of these therapies rather than post-hoc off-label usage.