ECMO and Impella Support Strategies as a Bridge to Surgical Repair of Post-Infarction Ventricular Septal Rupture.

Background and Objectives: Post-infarct ventricular septal rupture (PIVSR) continues to have significant morbidity and mortality, despite decreased prevalence. Impella and venoarterial extracorporeal membranous oxygenation (VA-ECMO) have been proposed as strategies to correct hemodynamic derangements and bridge patients to delayed operative repair when success rates are higher. This review places VA-ECMO and Impella support strategies in the context of bridging patients to successful PIVSR repair, with an additional case report of successful bridging with the Impella device. Materials and Methods: We report a case of PIVSR repair utilizing 14 days of Impella support. We additionally conducted a systematic review of contemporary literature to describe the application of VA-ECMO and Impella devices in the pre-operative period prior to surgical PIVSR correction. Expert commentary on the advantages and disadvantages of each of these techniques is provided. Results: We identified 19 studies with 72 patients undergoing VA-ECMO as a bridge to PIVSR repair and 6 studies with 11 patients utilizing an Impella device as a bridge to PIVSR repair. Overall, outcomes in both groups were better than expected from patients who were historically managed with medicine and balloon pump therapy, however there was a significant heterogeneity between studies. Impella provided for excellent left ventricular unloading, but did result in some concerns for reversal of shunting. VA-ECMO resulted in improved end-organ perfusion, but carried increased risks of device-related complications and requirement for additional ventricular unloading. Conclusions: Patients presenting with PIVSR in cardiogenic shock requiring a MCS bridge to definitive surgical repair continue to pose a challenge to the multidisciplinary cardiovascular team as the diverse presentation and management issues require individualized care plans. Both VA-ECMO and the Impella family of devices play a role in the contemporary management of PIVSR and offer distinct advantages and disadvantages depending on the clinical scenario. The limited case numbers reported demonstrate feasibility, safety, and recommendations for optimal management.

Tracheostomy decannulation to noninvasive positive pressure ventilation in congenital central hypoventilation syndrome.

PURPOSE: Noninvasive positive pressure ventilation (NPPV) may permit tracheostomy decannulation (TD) in patients with congenital central hypoventilation syndrome (CCHS) requiring nocturnal positive pressure ventilation via tracheostomy (PPV-T). There is limited evidence on optimal strategies for transitioning patients from PPV-T to NPPV. This study aimed to describe the clinical course and outcome of children with CCHS who underwent TD and transitioned from PPV-T to NPPV. METHODS: Retrospective review was conducted on patients with CCHS using nocturnal PPV-T who underwent TD to NPPV. The results of clinical evaluations, airway endoscopy, polysomnography, and clinical course leading to TD were analyzed. RESULTS: We identified 3 patients with CCHS aged 8-17 years who required PPV-T only during sleep. Patients underwent systematic multidisciplinary evaluations with a pediatric psychologist, pulmonologist, sleep physician, and otolaryngologist utilizing a TD algorithm. These included evaluation in the sleep clinic, NPPV mask fitting and desensitization, endoscopic airway evaluation, daytime tracheostomy capping, acclimatization to low-pressure NPPV, polysomnography with capped tracheostomy and NPPV titration, and if successful, TD. All patients underwent successful TD following optimal titration of NPPV during polysomnography. The duration to TD from decision to pursue NPPV was between 2.4 and 10.6 months, and the duration of hospitalization for TD was between 4 and 5 days. There were no NPPV-related complications; however, all patients required surgical closure of tracheocutaneous fistula. CONCLUSION: NPPV may be an effective and feasible option for patients with CCHS requiring PPV-T during sleep and permits TD. In patients with CCHS, a systematic multidisciplinary algorithm may optimize successful transition to NPPV and TD.

Recurrent apnoea and respiratory failure in an infant: congenital central hypoventilation syndrome with a novel PHOX2B gene variant.

A 20-day-old term infant presented with recurrent apnoea, lethargy and respiratory failure. Examination revealed episodes of apnoea and desaturation to 85% without any signs of respiratory distress requiring initiation of non-invasive positive pressure ventilation (NPPV). Capillary blood gas was indicative of respiratory acidosis and serum bicarbonate was elevated at 35 mmol/L. Chest radiograph, echocardiogram and evaluations for infectious aetiologies resulted normal. Due to inability to wean off NPPV with ensuing apnoea and desaturation, polysomnogram was performed and showed central and obstructive sleep apnoea, hypoxaemia and hypoventilation. Central apnoeas and hypoventilation were worse in non-rapid eye movement sleep. Paired-like homeobox 2B genetic studies showed a novel non-polyalanine repeat mutation (c.429+1G>A) establishing the diagnosis of congenital central hypoventilation syndrome (CCHS). Our case highlights the utility of polysomnography in the evaluation of term infants with apnoea. Although rare, clinicians should consider a diagnosis of CCHS in the evaluation of infants with apnoea and hypoventilation.

Improving the performance of peripheral arterial tonometry-based testing for the diagnosis of obstructive sleep apnea.

Outside sleep laboratory settings, peripheral arterial tonometry (PAT, eg, WatchPat) represents a validated modality for diagnosing obstructive sleep apnea (OSA). We have shown before that the accuracy of home sleep apnea testing by WatchPat 200 devices in diagnosing OSA is suboptimal (50%-70%). In order to improve its diagnostic performance, we built several models that predict the main functional parameter of polysomnography (PSG), Apnea Hypopnea Index (AHI). Participants were recruited in our Sleep Center and underwent concurrent in-laboratory PSG and PAT recordings. Statistical models were then developed to predict AHI by using robust functional parameters from PAT-based testing, in concert with available demographic and anthropometric data, and their performance was confirmed in a random validation subgroup of the cohort. Five hundred synchronous PSG and WatchPat sets were analyzed. Mean diagnostic accuracy of PAT was improved to 67%, 81% and 85% in mild, moderate-severe or no OSA, respectively, by several models that included participants' age, gender, neck circumference, body mass index and the number of 4% desaturations/hour. WatchPat had an overall accuracy of 85.7% and a positive predictive value of 87.3% in diagnosing OSA (by predicted AHI above 5). In this large cohort of patients with high pretest probability of OSA, we built several models based on 4% oxygen desaturations, neck circumference, body mass index and several other variables. These simple models can be used at the point-of-care, in order to improve the diagnostic accuracy of the PAT-based testing, thus ameliorating the high rates of misclassification for OSA presence or disease severity.

Performance of peripheral arterial tonometry-based testing for the diagnosis of obstructive sleep apnea in a large sleep clinic cohort.

STUDY OBJECTIVES: Peripheral arterial tonometry (PAT)-based technology represents a validated portable monitoring modality for the diagnosis of OSA. We assessed the diagnostic accuracy of PAT-based technology in a large point-of-care cohort of patients studied with concurrent polysomnography (PSG). METHODS: During study enrollment, all participants suspected to have OSA and tested by in-laboratory PSG underwent concurrent PAT device recordings. RESULTS: Five hundred concomitant PSG and WatchPat tests were analyzed. Median (interquartile range) PSG AHI was 18 (8-37) events/h and PAT AHI3% was 25 (12-46) events/h. Average bias was + 4 events/h. Diagnostic concordance was found in 42%, 41%, and 83% of mild, moderate, and severe OSA, respectively (accuracy = 53%). Among patients with PAT diagnoses of moderate or severe OSA, 5% did not have OSA and 19% had mild OSA; in those with mild OSA, PSG showed moderate or severe disease in 20% and no OSA in 30% of patients (accuracy = 69%). On average, using a 3% desaturation threshold, WatchPat overestimated disease prevalence and severity (mean + 4 events/h) and the 4% threshold underestimated disease prevalence and severity by -6 events/h. CONCLUSIONS: Although there was an overall tendency to overestimate the severity of OSA, a significant percentage of patients had clinically relevant misclassifications. As such, we recommend that patients without OSA or with mild disease assessed by PAT undergo repeat in-laboratory PSG. Optimized clinical pathways are urgently needed to minimize therapeutic decisions instituted in the presence of diagnostic uncertainty.

Respiratory Syncytial Virus Associated Myocarditis Requiring Venoarterial Extracorporeal Membrane Oxygenation.

Severe fulminant myocarditis causing cardiogenic shock can be a rapidly progressing, life threatening condition. Respiratory syncytial virus (RSV) is a very rare infectious culprit infrequently described in medical literature as a cause of myocarditis, particularly in adults. We present a case of acute fulminant myocarditis in a patient with PCR positive RSV infection requiring venoarterial extracorporeal membrane oxygenation (VA-ECMO).