Maternal and Fetal Outcomes in Pulmonary Hypertension During Pregnancy: A Contemporary Nationwide Analysis.

Pulmonary hypertension (PH) disproportionately affects women, presenting challenges during pregnancy. Historically, patients with PH are advised to avoid pregnancy; however, recent reports have indicated that the incidence of adverse events in pregnant patients with PH may be lower than previously reported. We conducted a retrospective cohort study in pregnant patients with PH using the National Readmission Database from January 1, 2016, to December 31, 2020. PH was categorized according to the World Health Organization classification. Primary end points include maternal mortality and 30-day nonelective readmission rate. Other adverse short-term maternal (cardiovascular and obstetric) and fetal outcomes were also analyzed. Of 9,922,142 pregnant women, 3,532 (0.04%) had PH, with Group 1 PH noted in 1,833 (51.9%), Group 2 PH in 676 (19.1%), Group 3 PH in 604 (17.1%), Group 4 PH in 23 (0.7%), Group 5 PH in 98 (2.8%), and multifactorial PH in 298 (8.4%). PH patients exhibited higher rates of adverse cardiovascular events (15.7% vs 0.3% without PH, p <0.001) and mortality (0.9% vs 0.01% without PH, p <0.001). Mixed PH and Group 2 PH had the highest prevalence of adverse cardiovascular events in the World Health Organization PH groups. Patients with PH had a significantly higher nonelective 30-day readmission rate (10.4% vs 2.3%) and maternal adverse obstetric events (24.2% vs 9.1%) compared with those without PH (p <0.001) (Figure 1). In conclusion, pregnant women with PH had significantly higher adverse event rates, including in-hospital maternal mortality (85-fold), compared with those without PH.

A center experience with lung transplantation for COVID-19 ARDS.

COVID-19 can cause irreversible lung damage from acute respiratory distress syndrome (ARDS), chronic respiratory failure associated with post COVID-19 de novo fibrosis or worsening of an underlying fibrotic lung disease. Pregnant women are at increased risk for invasive mechanical ventilation, extracorporeal membrane oxygenation, and death. The Centers for Disease Control and Prevention reported more than 22,000 hospitalizations and 161 deaths for COVID-19 in pregnant women. Between August 2020 and September 2021, five patients underwent bilateral lung transplant (LT) for COVID-19 ARDS at the Henry Ford Hospital in Detroit, Michigan. De-identified demographics data, clinical characteristics, perioperative challenges, explanted lung pathology, and post-transplant outcomes are described. In post-hospitalization follow-up (median survival 273 days), we see improving endurance and excellent lung function. One patient did not survive to hospital discharge and succumbed to complications 5 months after LT. We report the first cases of bilateral LT in two postpartum women.

Serious illness conversations in pulmonary hypertension.

Pulmonary arterial hypertension has evolved from a fatal disease with few treatment options to a chronic condition with improved survival. This improvement is possible through development of effective therapies as well as the expansion of risk stratification scores to assist clinical decision making. Despite improved disease control, quality of life, and overall prognosis, many challenges remain. The treatment itself is burdensome, with significant impact on quality of life. Many patients with pulmonary arterial hypertension still present with advanced, often end-stage disease. Increased use of mechanical circulatory support and catheter-based interventions have expanded use of extracorporeal life support and right ventricle assist devices. For these reasons as well as the long-term relationships pulmonary hypertension physicians have with patients and their families, navigating the course of the illness in a considered, proactive way is essential. Understanding individual goals and revisiting them as they change over time requires comfort with the conversation itself. There are many barriers and challenges to having effective, compassionate conversations in the clinical setting with time constraints being the most often cited. Compressed visits are necessarily focused on the clinical aspects, therapy and medication adherence and tolerance. Clinicians are sometimes wary of diminishing hope in the face of ongoing treatment. Having sufficient experience and comfort with these discussions can be empowering. In this paper, we discuss the challenges involved and propose a framework to assist in incorporating these discussions into clinical care.

A 24-Year-Old Woman With Precipitous Respiratory Failure After Lung Transplantation.

CASE PRESENTATION: A 24-year-old woman with ΔF508/Y1092X cystic fibrosis (CF) complicated by severe obstructive lung disease (FEV1 of 30% predicted) was admitted for IV antibiotics for planned sinus surgery resulting from severe chronic sinusitis causing frequent exacerbations and declining lung function. She had persistent airway infection with multidrug-resistant Pseudomonas aeruginosa, methicillin-resistant Staphylococcus aureus, and growth of a fungus presumed to be an airway colonizer, identified as Stephanoascus ciferrii 1 year before presentation. Two days after surgery, she developed acute respiratory failure requiring mechanical ventilation. On day 4 of mechanical ventilation, venovenous-extracorporeal membrane oxygenation (VV-ECMO) was initiated for refractory respiratory failure. The following day, she was listed for bilateral lung transplant and was transplanted 4 days later. Following transplantation, she was decannulated from ECMO; however, over the next 12 hours, oxygenation deteriorated requiring reinstitution of VV-ECMO for presumed severe primary graft dysfunction. Despite treatment with broad spectrum antimicrobial coverage with piperacillin/tazobactam, ciprofloxacin, linezolid, micafungin, voriconazole, and ganciclovir, she failed to improve and developed complex bilateral pleural effusions.

An unusual case of interstitial lung disease in a patient with cardiopulmonary syndrome as the initial presentation of Erdheim-Chester disease.

Erdheim-Chester disease (ECD) is a very rare disorder with only approximately 600 cases reported in the literature. ECD has been recently reclassified as a histiocytic dendritic cell neoplasm. The clinical spectrum ranges from asymptomatic tissue accumulation of histiocytes to invasive tissue infiltration, which can cause fulminant multisystem failure. It typically presents with bone pain and constitutional symptoms. Extraosseous manifestations are not uncommon. ECD-associated interstitial lung disease has been described in 20%-35% of patients. Diagnosis is primarily by tissue biopsy and immunohistochemistry showing xanthogranulomas composed of foamy histiocytes that stain positive for CD68, CD14 and CD163 and negative for CD1á and langerin. We report a case of ECD in a young man with cardiopulmonary involvement who presented with haemoptysis and dyspnoea.

Early postoperative management after lung transplantation: Results of an international survey.

INTRODUCTION: Little data exist regarding optimal therapeutic strategies postoperatively after lung transplant (LTx). Current practice patterns rely on expert opinion and institutional experience resulting in nonuniform postoperative care. To better define current practice patterns, an international survey of LTx clinicians was conducted. METHODS: A 30-question survey was sent to transplant clinicians via email to the International Society of Heart and Lung Transplantation open forum mailing list and directly to the chief transplant surgeon and pulmonologist of all LTx centers in the United States. RESULTS: Fifty-two clinicians representing 10 countries responded to the survey. Sedatives use patterns included: opiates + propofol (57.2%), opiates + dexmedetomidine (18.4%), opiates + intermittent benzodiazepines (14.3%), opiates + continuous benzodiazepines (8.2%), and opiates alone (2%). About 40.4% reported no formal sedation scale was followed and 13.5% of programs had no formal policy on sedation and analgesia. A lung protective strategy was commonly employed, with 13.8%, 51.3%, and 35.9% of respondents using tidal volumes of <6 mL/kg ideal body weight (IBW), 6 mL/kg IBW, and 8 mL/kg IBW, respectively. CONCLUSION: Practice patterns in the early postoperative care of lung transplant recipients differ considerably among centers. Many of the reported practices do not conform to consensus guidelines on management of critically ill patients.

Retroperitoneal amyloidosis as the presenting manifestation of Waldenstrom's macroglobulinaemia.

Retroperitoneal amyloidosis has been described in a few case reports and is typically a secondary manifestation of inflammation or malignancy. We present the case of a 69-year-old man who presented with a large pleural effusion and an incidental retroperitoneal mass in the CT imaging. Further investigation confirmed the diagnosis of amyloid amyloidosis involving the retroperitoneum as well as a concurrent Waldenstrom's macroglobulinaemia. Chemotherapy consistent of drugs active against both the lymphoid and plasma cell components of the disease is the proposed therapy for Waldenstrom's macroglobulinaemia in those patients amenable to receive chemotherapy and can make a difference in the survival.