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Journal : Narra J

Challenges in diagnosing and treating Liddle syndrome in resource-limited settings: A case report from Indonesia Prabowo, Nurhasan A.; Putranto, Wachid; Myrtha, Risalina; Ardyanto, Tonang D.; Gautama, Coana S.; Wulandari, Evi L.; Hermawati, Berty D.; Putri, Desy P.; Ramadhani, Artika; Dewi, Herlina K.
Narra J Vol. 4 No. 3 (2024): December 2024
Publisher : Narra Sains Indonesia

Show Abstract | Download Original | Original Source | Check in Google Scholar | DOI: 10.52225/narra.v4i3.1000

Abstract

Liddle syndrome, a rare form of monogenic hypertension, poses significant diagnostic and therapeutic challenges due to its phenotypic variability and the need for genetic testing. The rarity of the condition, coupled with the limited availability of first-line treatments such as epithelial sodium channel (ENaC) blockers, makes this case report particularly urgent and novel, highlighting alternative management strategies in resource-limited settings. The aim of this case report was to present the diagnostic challenges, therapeutic strategies, and clinical outcomes of a patient with Liddle syndrome who did not have access to ENaC blockers, emphasizing the importance of early recognition and personalized treatment. A 35-year-old female presented with resistant hypertension (190/100 mmHg) and bilateral limb weakness. Laboratory results revealed persistent hypokalemia, hypernatremia, and metabolic alkalosis. Low aldosterone levels, alongside clinical and family history, led to the diagnosis of Liddle syndrome. Genetic testing was not conducted due to resource limitations, and ENaC blockers were unavailable. The patients were managed with a combination of alternative antihypertensive agents, potassium supplementation, and a low-sodium diet. Although this approach led to modest improvements in blood pressure and motor strength, persistent hypokalemia and hypernatremia underscored the suboptimal control of the syndrome's underlying pathophysiology in the absence of ENaC blockers. This case highlights the challenges faced in resource-limited settings and the need for innovative strategies to manage rare conditions like Liddle syndrome. Liddle syndrome's diagnostic and therapeutic challenges underscore the critical importance of early recognition and access to targeted therapies. In the absence of ENaC blockers, alternative treatment strategies can provide some benefit, but they often fall short of optimal management. This case emphasizes the need for enhanced clinical awareness, improved access to genetic testing, and the development of personalized treatment approaches to achieve better patient outcomes.
Zero-fluoroscopy versus fluoroscopy-guided catheter ablation in ventricular arrhythmia: A systematic review and meta-analysis Irnizarifka, Irnizarifka; Tristan, Christopher D.; Wijayanto, Matthew A.; Myrtha, Risalina; Modesty, Kyra; Rahma, Annisa A.; Budiono, Enrico A.; Rahman, Awalil R. K.; Hamka, Muhammad Farid; Ilyas, Muhana F.
Narra J Vol. 5 No. 2 (2025): August 2025
Publisher : Narra Sains Indonesia

Show Abstract | Download Original | Original Source | Check in Google Scholar | DOI: 10.52225/narraj.v5i2.2094

Abstract

Catheter ablation has been the go-to treatment for ventricular arrhythmia, with traditional fluoroscopy-guided and non-zero fluoroscopy (NZF) catheter ablation posing high radiation risk for operators and patients. Zero-fluoroscopy technique offers elimination of radiation risk; however, its efficacy and safety in ventricular arrhythmia patients are not well explored. The aim of this study was to systematically evaluate the effectiveness, safety, and feasibility of zero-fluoroscopy ablation on ventricular arrhythmia patients. This study only included relevant studies comparing zero-fluoroscopy and NZF in ventricular arrhythmia ablation that were identified from Scopus, PubMed, and ScienceDirect (up to June 20, 2024). The quality of the study was assessed using the ROBINS-I tool, and the meta-analysis was conducted using a random-effect model. Out of 383 studies found, nine cohort studies were included with 1.408 patients. There was no significant difference in the acute procedural success rate of the zero-fluoroscopy and NZF (relative risk: 1.01; 95%CI: 0.95–1.07; p=0.69), with a similar recurrence rate (p=0.88; for four studies; n=374), and comparable procedural time (mean difference: -19.22 minutes; 95%CI: -41.16–2.72; p=0.09). Adverse events such as pericardial effusion, pseudoaneurysm, and hematoma were similar between zero-fluoroscopy and NZF. Overall, zero-fluoroscopy catheter ablation has demonstrated non-inferiority as a treatment option for ventricular arrhythmia ablation. As zero-fluoroscopy eliminates radiation risk without compromising procedural efficacy, zero-fluoroscopy has the potential to become a widely adopted approach for catheter ablation in ventricular arrhythmia.