Early, Postnatal Pulmonary Hypertension Severity Predicts Inpatient Outcomes in Congenital Diaphragmatic Hernia

Neonatology ◽  
2021 ◽  
pp. 1-8
Author(s):  
Dalya Munves Ferguson ◽  
Vikas S. Gupta ◽  
Pamela A. Lally ◽  
Matias Luco ◽  
KuoJen Tsao ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Congenital Diaphragmatic Hernia ◽  
Diaphragmatic Hernia ◽  
Life Support ◽  
Extracorporeal Life Support ◽  
Systolic Pressure ◽  
Right Ventricular Systolic Pressure ◽  
Ventricular Systolic Pressure ◽  
Inpatient Outcomes ◽  
Increased Risk

<b><i>Introduction:</i></b> Pulmonary hypertension (PH) is the major pathophysiologic consequence of congenital diaphragmatic hernia (CDH). We aimed to evaluate the association between early CDH-associated PH (CDH-PH) and inpatient outcomes. <b><i>Methods:</i></b> The CDH Study Group registry was queried for infants born 2015–2019 with echocardiograms before 48h of life. PH was categorized using echocardiographic findings: none, mild (right ventricular systolic pressure &#x3c;2/3 systemic), moderate (between 2/3 systemic and systemic), or severe (supra-systemic). Univariate and multivariate analyses were performed. Adjusted Poisson regression was used to assess the primary composite outcome of mortality or oxygen support at 30 days. <b><i>Results:</i></b> Of 1,472 patients, 86.5% had CDH-PH: 13.9% mild (<i>n</i> = 193), 44.4% moderate (<i>n</i> = 631), and 33.2% severe (<i>n</i> = 468). On adjusted analysis, the primary outcome of mortality or oxygen support at 30 days occurred more frequently in infants with moderate (incidence rate ratio [IRR] 1.8, 95% confidence interval [CI], 1.2–2.6) and severe CDH-PH (IRR 2.0, 95% CI, 1.3–2.9). Extracorporeal life support (ECLS) utilization was associated only with severe CDH-PH after adjustment (IRR 1.8, 95% CI, 1.0–3.3). <b><i>Discussion/Conclusion:</i></b> Early, postnatal CDH-PH is independently associated with increased risk for mortality or oxygen support at 30 days and utilization of ECLS. Early echocardiogram is a valuable prognostic tool for early, inpatient outcomes in neonates with CDH.

scholarly journals ECMO em Recém-nascidos com Hérnia Diafragmática Congénita: A Experiência de um Centro de Referência de ECMO em Portugal

2020 ◽  
Vol 33 (12) ◽  
pp. 819
Author(s):  
Mariana Miranda ◽  
Francisco Abecasis ◽  
Sofia Almeida ◽  
Erica Torres ◽  
Leonor Boto ◽  
...  
Keyword(s):  
Congenital Diaphragmatic Hernia ◽  
Diaphragmatic Hernia ◽  
Life Support ◽  
Extracorporeal Life Support ◽  
Ventilatory Support ◽  
Pediatric Intensive Care ◽  
Cycle Duration ◽  
Extracorporeal Life Support Organization ◽  
Support Organization ◽  
Technological Advances

Introduction: The use of extracorporeal membrane oxygenation (ECMO) is considered by many authors as one of the most important technological advances in the care of newborns with congenital diaphragmatic hernia. The main objective of this study was to report the experience of a Portuguese ECMO center in the treatment of congenital diaphragmatic hernia.Material and Methods: Descriptive retrospective study of newborns with congenital diaphragmatic hernia requiring ECMO support in a Pediatric Intensive Care Unit from January 2012 to December 2019. Data collection using the Extracorporeal Life Support Organization registration and unit data base.Results: Fourteen newborns were included, all with left congenital diaphragmatic hernia, in a total of 15 venoarterial ECMO cycles. The median gestational age was 38 weeks and the median birth weight was 2.950 kg. Surgical repair was performed before entry into ECMO in six, during in seven and after in one newborn. The average age at placement was 3.3 days and the median cycle duration was 16 days. Prior to ECMO, all newborns had severe hypoxemia and acidosis despite optimized ventilatory support, with nitric oxide and inotropic therapy. After 24 hours on ECMO, there was correction of acidosis, improvement of oxygenation and hemodynamic stability. All cycles presented mechanical complications, the most frequent being the presence of clots in the circuit. The most frequent physiological complications were hemorrhagic and embolic (three newborns suffered an ischemic stroke during the cycle). Five newborns (35.7%) died, all associated with complications (two strokes, two massive bleedings and one accidental decannulation). Chronic lung disease, poor weight gain and psychomotor developmental delay were the most frequent long-term morbidities.Discussion: Despite technological advances in respiratory care and improved safety of the ECMO technique, the management of these newborns is complex and there are still several open questions, including the appropriate selection of patients, the best approach and time for surgical correction, and the treatment of pulmonary hypertension in the presence of persistent fetal shunts.Conclusion: Survival rate was higher than reported in 2017 Extracorporeal Life Support Organization report (64% versus 50%). Mechanical and hemorrhagic complications were very frequent.

Abstract 14666: Association Between Right Ventricular Systolic Pressure and Health Status Following Transcatheter Aortic Valve Replacement

Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Rahul Myadam ◽  
Ali O Malik ◽  
Matthew Pflederer ◽  
Kensey Gosch ◽  
Suzanne V Arnold ◽  
...  
Keyword(s):  
Health Status ◽  
Transcatheter Aortic Valve Replacement ◽  
Mixed Model ◽  
Linear Mixed Model ◽  
Systolic Pressure ◽  
Right Ventricular Systolic Pressure ◽  
Ventricular Systolic Pressure ◽  
Transcatheter Aortic Valve ◽  
Increased Risk ◽  
Before And After

Introduction: Pulmonary hypertension (PH) was shown in multiple studies to be associated with an increased risk of mortality after transcatheter aortic valve replacement (TAVR). However, it is unclear if echocardiogram derived right ventricular systolic pressure (RVSP) is associated with health status outcomes in surviving patients after TAVR. We explored for an association between baseline RVSP and quality of life in patients before and after undergoing TAVR. Methods: We estimated RVSP by echocardiography using the modified Bernoulli equation in a single-center cohort of patients undergoing TAVR from 2012-2017 . Disease-specific health status was assessed at baseline and 1-month and 12-months after TAVR with the Kansas City Cardiomyopathy Questionnaire-Overall Summary Score (KCCQ-OS). We then explored the association between baseline RVSP and KCCQ-OS before and after TAVR using a linear mixed model with an interaction for time and baseline RVSP and adjusted for baseline mitral valve regurgitation and systolic blood pressure. Results: Among 485 patients who underwent TAVR (mean age 81.7±7.9 years, 54.8% men), baseline RVSP was 42±15 mmHg, and 73% had RVSP >34 mmHg. After TAVR, mean RVSP decreased to 37±13 mmHg at 1 month and 36±14 mmHg at 12 months. Baseline KCCQ-OS was 46±25 and improved to 66.9±23.6 at 1 month and 69.5± 22.6 at 12 months. In the linear mixed model, there was a significant cross-sectional association between baseline RVSP and baseline KCCQ-OS, with higher RVSP associated with worse health status. However, baseline RVSP was not significantly associated with KCCQ-OS at 1 month or 12 months (Figure). Conclusions: RVSP is not associated with worse health status after TAVR. This suggests that while patients with high RVSP are at an increased risk for mortality after TAVR, surviving patients appear to have similar health status as those with normal RVSP.

scholarly journals Chronic Hypoxia Decreases Endothelial Connexin 40, Attenuates Endothelium‐Dependent Hyperpolarization–Mediated Relaxation in Small Distal Pulmonary Arteries, and Leads to Pulmonary Hypertension

2020 ◽  
Vol 9 (24) ◽  
Author(s):  
Rui Si ◽  
Qian Zhang ◽  
Jody Tori O. Cabrera ◽  
Qiuyu Zheng ◽  
Atsumi Tsuji‐Hosokawa ◽  
...  
Keyword(s):  
Endothelial Cells ◽  
Pulmonary Hypertension ◽  
Right Ventricular ◽  
Knockout Mice ◽  
Chronic Hypoxia ◽  
Systolic Pressure ◽  
Right Ventricular Systolic Pressure ◽  
Ventricular Systolic Pressure ◽  
Pulmonary Endothelial Cells ◽  
Endothelium Dependent Relaxation

Background Abnormal endothelial function in the lungs is implicated in the development of pulmonary hypertension; however, there is little information about the difference of endothelial function between small distal pulmonary artery (PA) and large proximal PA and their contribution to the development of pulmonary hypertension. Herein, we investigate endothelium‐dependent relaxation in different orders of PAs and examine the molecular mechanisms by which chronic hypoxia attenuates endothelium‐dependent pulmonary vasodilation, leading to pulmonary hypertension. Methods and Results Endothelium‐dependent relaxation in large proximal PAs (second order) was primarily caused by releasing NO from the endothelium, whereas endothelium‐dependent hyperpolarization (EDH)–mediated vasodilation was prominent in small distal PAs (fourth–fifth order). Chronic hypoxia abolished EDH‐mediated relaxation in small distal PAs without affecting smooth muscle–dependent relaxation. RNA‐sequencing data revealed that, among genes related to EDH, the levels of Cx37 , Cx40 , Cx43 , and IK were altered in mouse pulmonary endothelial cells isolated from chronically hypoxic mice in comparison to mouse pulmonary endothelial cells from normoxic control mice. The protein levels were significantly lower for connexin 40 (Cx40) and higher for connexin 37 in mouse pulmonary endothelial cells from hypoxic mice than normoxic mice. Cx40 knockout mice exhibited significant attenuation of EDH‐mediated relaxation and marked increase in right ventricular systolic pressure. Interestingly, chronic hypoxia led to a further increase in right ventricular systolic pressure in Cx40 knockout mice without altering EDH‐mediated relaxation. Furthermore, overexpression of Cx40 significantly decreased right ventricular systolic pressure in chronically hypoxic mice. Conclusions These data suggest that chronic hypoxia‐induced downregulation of endothelial Cx40 results in impaired EDH‐mediated relaxation in small distal PAs and contributes to the development of pulmonary hypertension.

Effect of very delayed repair of congenital diaphragmatic hernia on survival and extracorporeal life support use

Surgery ◽  
1996 ◽  
Vol 120 (4) ◽  
pp. 766-773 ◽  
Author(s):  
Craig A. Reicker ◽  
Ronald B. Hirschl ◽  
Robert Schumacher ◽  
James D. Geiger ◽  
Charles Cox ◽  
...  
Keyword(s):  
Congenital Diaphragmatic Hernia ◽  
Diaphragmatic Hernia ◽  
Life Support ◽  
Extracorporeal Life Support ◽  
Delayed Repair

Survival Benefit Associated With the Use of Extracorporeal Life Support for Neonates With Congenital Diaphragmatic Hernia

2020 ◽  
Vol Publish Ahead of Print ◽  
Author(s):  
Tim Jancelewicz ◽  
Max R. Langham ◽  
Mary E. Brindle ◽  
Zachary E. Stiles ◽  
Pamela A. Lally ◽  
...  
Keyword(s):  
Congenital Diaphragmatic Hernia ◽  
Diaphragmatic Hernia ◽  
Survival Benefit ◽  
Life Support ◽  
Extracorporeal Life Support

scholarly journals Extracorporeal Life Support in Patients with Congenital Diaphragmatic Hernia: How Long Should We Treat?

2014 ◽  
Vol 218 (4) ◽  
pp. 808-817 ◽  
Author(s):  
David W. Kays ◽  
Saleem Islam ◽  
Douglas S. Richards ◽  
Shawn D. Larson ◽  
Joy M. Perkins ◽  
...  
Keyword(s):  
Congenital Diaphragmatic Hernia ◽  
Diaphragmatic Hernia ◽  
Life Support ◽  
Extracorporeal Life Support

scholarly journals Prevalence, Incidence and Associates of Pulmonary Hypertension Complicating Type 2 Diabetes: Insights from the Fremantle Diabetes Study Phase 2 and National Echocardiographic Database of Australia

2021 ◽  
Vol 10 (19) ◽  
pp. 4503
Author(s):  
Nishant Nundlall ◽  
David Playford ◽  
Geoff Strange ◽  
Timothy M. E. Davis ◽  
Wendy A. Davis
Keyword(s):  
Type 2 Diabetes ◽  
Pulmonary Hypertension ◽  
Systolic Pressure ◽  
Study Phase ◽  
Phase 2 ◽  
Right Ventricular Systolic Pressure ◽  
Ventricular Systolic Pressure ◽  
Multivariable Analyses ◽  
The Relationship

There is a paucity of epidemiologic data examining the relationship between pulmonary hypertension (PH) and diabetes. The aim of this study was to determine prevalence, incidence and associates of PH complicating type 2 diabetes. Data from 1430 participants (mean age 65.5 years, 51.5% males) in the Fremantle Diabetes Study Phase 2 (FDS2) were linked with the National Echocardiographic Database of Australia (NEDA) to ascertain the prevalence and incidence of PH (estimated right ventricular systolic pressure (eRVSP) >30 mmHg as a new suggested threshold or the conventional >40 mmHg) over a 12-year period. PH prevalence in FDS2 was compared with that in NEDA overall and a geographically close sub-population. Multivariable analyses identified associates of prevalent/incident PH in the FDS2 cohort. Of 275 FDS2 patients (19.2%) with pre-entry echocardiography, 90 had eRVSP >30 mmHg and 35 had eRVSP >40 mmHg (prevalences 32.7% (95% CI 27.3–38.7%) and 12.7% (9.1–17.4%), respectively), rates that are 35–50% greater than national/local NEDA general population estimates. Moreover, 70 (5.0%) and 123 (9.2%) FDS2 participants were identified with incident PH at the respective eRVSP thresholds (incidence (95% CI) 7.6 (6.0–9.7) and 14.2 (11.8–17.0)/1000 person-years), paralleling data from recognised high-risk conditions such as systemic sclerosis. The baseline plasma N-terminal pro-brain natriuretic peptide concentration was the strongest independent associate of prevalent/incident PH. Approximately 1 in 8 people with type 2 diabetes have PH using the eRVSP >40 mmHg threshold. Its presence should be considered as part of regular clinical assessment of individuals with type 2 diabetes.

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