Pulmonary Versus Systemic Outflow Obstruction in Functionally Univentricular Hearts with Isomerism: An Observation

SummaryAccessory atrioventricular valvar tissue is uncommon and, on occasion, has been identified as a cause of ventricular outflow obstruction. Accessory tricuspid valvar tissue has been reported to cause subpulmonary obstruction but infrequently has accessory tissue arising from the mitral valve been associated with obstruction. This paper reports two cases of subvalvar obstruction; the first in association with a ventricular septal defect causing subaortic obstruction and the other in association with congenitally corrected transposition and a ventricular septal defect, causing subpulmonary obstruction.

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Solid Swallow Examination During High Resolution Manometry and EGJ-Distensibility Help Identify Esophageal Outflow Obstruction in Non-obstructive Dysphagia

Dysphagia ◽

10.1007/s00455-021-10260-0 ◽

2021 ◽

Author(s):

Fritz Ruprecht Murray ◽

Lara Maria Fischbach ◽

Valeria Schindler ◽

Larissa Schnurre ◽

Juliane Marie Hente ◽

...

Keyword(s):

High Resolution ◽

Diagnostic Yield ◽

Diagnostic Value ◽

Control Group ◽

Clinical Response Rate ◽

Interventional Treatment ◽

Outflow Obstruction ◽

Motility Disorders ◽

High Resolution Manometry ◽

Solid Swallows

AbstractSingle water swallow (SWS) high-resolution manometry (HRM) may miss relevant esophageal motility disorders. Solid test meal (STM) during HRM and lately the functional lumen imaging probe (FLIP) have been shown to be of diagnostic value in the assessment of motility disorders. We aimed to assess the diagnostic yield of STM and FLIP in non-obstructive dysphagia (NOD). Patients assessed for dysphagia with both HRM and FLIP between April 2016 and August 2019 were analyzed for signs of non-obstructive EGJ outflow obstruction (EGJOO) according to Chicago Classification 3.0 (CCv3) and CC adapted for the use with solid swallows (CC-S), followed by an individual group-specific analysis. Five subjects without dysphagia served as control group. Standard HRM- and FLIP-values as well as esophagograms and Eckardt Scores were analyzed. Forty-two patients were identified (male/female, 14/36, median age 62). Twenty-five (59.5%) were diagnosed with EGJOO during STM only (= SWS-negative patients; CC-S). The EGJ distensibility index (EGJ-DI) of symptomatic patients was significantly lower compared to the control group (p = 0.006). EGJ-DI was < 3mm2/mmHg in 67% and 88% of patients diagnosed according to CC-S and CCv3, respectively. The IRP during STM showed a significant association to the corresponding EGJ-DI values (p < 0.001). Seventy-six percent of patients received treatment because of additional STM evaluation with a favorable clinical response rate of 89%. STM and FLIP identify EGJOO in symptomatic patients with normal SWS during HRM. STM resembles an inexpensive and clinically meaningful option to diagnose motility disorders and helps to select patients for interventional treatment.

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Physiological and pathological roles of the accommodation response in lower esophageal sphincter relaxation during wet swallows

Scientific Reports ◽

10.1038/s41598-021-87052-x ◽

2021 ◽

Vol 11 (1) ◽

Author(s):

Kazumasa Muta ◽

Eikichi Ihara ◽

Shohei Hamada ◽

Hiroko Ikeda ◽

Masafumi Wada ◽

...

Keyword(s):

Clinical Practice ◽

High Resolution ◽

Observational Study ◽

Lower Esophageal Sphincter ◽

Relaxation Function ◽

Esophagogastric Junction ◽

Prospective Observational Study ◽

Outflow Obstruction ◽

High Resolution Manometry ◽

Esophageal Sphincter

AbstractThe preparatory accommodation response of lower esophageal sphincter (LES) before swallowing is one of the mechanisms involved in LES relaxation during wet swallows, however, the physiological and/or pathological roles of LES accommodation remain to be determined in humans. To address this problem, we conducted a prospective observational study of 38 patients with normal high-resolution manometry (HRM) and 23 patients with idiopathic esophagogastric junction outflow obstruction (EGJOO) to assess dry and wet swallows. The LES accommodation measurement was proposed for practical use in evaluating the LES accommodation response. Although swallow-induced LES relaxation was observed in both dry and wet swallows, LES accommodation (6.4, 3.1–11.1 mmHg) was only observed in wet swallows. The extent of LES accommodation was impaired in idiopathic EGJOO (0.6, − 0.6–6 mmHg), and the LES accommodation measurement of patients with idiopathic EGJOO (36.8, 29.5–44.3 mmHg) was significantly higher in comparison to those with normal HRM (23.8, 18–28.6 mmHg). Successful LES relaxation in wet swallowing can be achieved by LES accommodation in combination with swallow-induced LES relaxation. Impaired LES accommodation is characteristic of idiopathic EGJOO. In addition to the IRP value, the LES accommodation measurement may be useful for evaluating the LES relaxation function in clinical practice.

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Oxygen Saturation and Perfusion Index-Based Enhanced Critical Congenital Heart Disease Screening

American Journal of Perinatology ◽

10.1055/s-0039-1685445 ◽

2019 ◽

Vol 37 (02) ◽

pp. 158-165 ◽

Cited By ~ 3

Author(s):

Heather Siefkes ◽

Laura Kair ◽

Daniel J. Tancredi ◽

Brian Vasquez ◽

Lorena Garcia ◽

...

Keyword(s):

Congenital Heart Disease ◽

Heart Disease ◽

Oxygen Saturation ◽

Congenital Heart ◽

Perfusion Index ◽

Outflow Obstruction ◽

Critical Congenital Heart Disease ◽

Healthy Infants ◽

Optimal Screening ◽

To Come

Objective To determine if addition of perfusion index (PIx) to oxygen saturation (SpO2) screening improves detection of critical congenital heart disease (CCHD) with systemic outflow obstruction. Study Design We determined screening thresholds for PIx and applied these to a cohort of newborns with and without congenital heart disease (CHD). Results A total of 123 normal and 21 CHD newborns (including five with critical systemic outflow obstruction) were enrolled. Four of these five critical systemic obstruction subjects passed SpO2-based screen. Four out of these five subjects failed PIx-based screen. The sensitivity for detection of systemic obstruction CCHD when compared with healthy infants increased from 20% (95% confidence interval [CI]: 1–72%) with SpO2 screening alone to 80% (95% CI: 28–100%) with combined SpO2-PIx screen. However, 2.44% of normal infants failed PIx screen. Conclusion Addition of PIx to SpO2 screening may detect additional cases of CCHD and further research is necessary to come up with optimal screening thresholds.

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