Letter to the Editor
Malignant ventricular arrhythmias in a patient with Gitelman syndrome
Arritmias ventriculares malignas en un paciente con síndrome de Gitelman
Adrián Riaño Ondiviela
, Daniel Meseguer González, Jose Ramón Ruiz Arroyo
Corresponding author
Servicio de Cardiología, Hospital Clínico Universitario Lozano Blesa, Zaragoza, Spain
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"identificador" => "cor0005" ] ] ] 1 => array:2 [ "nombre" => "Daniel" "apellidos" => "Meseguer González" ] 2 => array:2 [ "nombre" => "Jose Ramón" "apellidos" => "Ruiz Arroyo" ] ] "afiliaciones" => array:1 [ 0 => array:2 [ "entidad" => "Servicio de Cardiología, Hospital Clínico Universitario Lozano Blesa, Zaragoza, Spain" "identificador" => "aff0005" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Arritmias ventriculares malignas en un paciente con síndrome de Gitelman" ] ] "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Gitelman syndrome is a tubule disease caused by defects in the sodium–chloride symporter located in the distal convoluted tubule of the nephron. It is a rare genetic disorder, with an autosomal recessive inheritance pattern, which causes affected patients to have low potassium levels and, unlike Bartter syndrome, hypomagnesemia and hypocalciuria.<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> Although its phenotypic expression is highly variable and its diagnosis is often incidental or based on non-specific symptoms, the electrolyte disturbances caused by this syndrome can often cause malignant ventricular arrhythmias.</p><p id="par0010" class="elsevierStylePara elsevierViewall">In this paper we describe the case of a 31-year-old, male, active smoker without a previous relevant medical history. He did not report a use of laxatives or diuretics. He complained of asthenia, generalized weakness, and palpitations of several weeks evolution that had progressed gradually for several weeks and intensified over the last few hours, which is why he decided to visit the Emergency Department. A physical examination revealed muscle hypotonia and shallow breathing. Blood pressure: 113/55 mmHg. Heart rate: 55 bpm. Respiratory rate: 12 bpm. Basal oxygen saturation: 96%. A cardiac auscultation revealed rhythmic heart sounds, without murmurs nor signs suggestive of heart failure. However, additional monitoring in the Emergency Department recorded several bursts of non-sustained ventricular tachycardia. Emergency blood work detected metabolic alkalosis compensated by a pH of 7.42 and bicarbonate (CO<span class="elsevierStyleInf">3</span>H) levels of 31 mmol/L, creatinine levels of 1.44 mg/dl, hypomagnesemia of 0.87 mmol/L (normal range: 1.6–2.6), very intense hypokalemia of 1.38 mEq/L (3.5–5.1), and an otherwise normal blood count and electrolyte panel. Because a surface electrocardiogram (ECG) documented a QT interval corrected by Bazett's formula (QTc) of 560 ms (normal value in men <440 ms), intravenous treatment with potassium chloride and magnesium sulfate was started. Shortly after starting the perfusion, the patient developed hemodynamic instability due to a torsades de pointes rhythm, owing to which electrical cardioversion at 200 J was required. In the following hours, he continued to experience different types of ventricular arrhythmias, such that a sustained perfusion of high doses of potassium and magnesium was needed to finally achieve membrane stabilization and, therefore, reduce the arrhythmic load.</p><p id="par0015" class="elsevierStylePara elsevierViewall">During the hypokalemia study, decreased calcium excretion was detected in a 24-h urine sample (44 mg/24 h; normal range: 100–250), together with increased sodium (360 mEq/L; normal range: 50–210), magnesium (180 mg/24 h; normal range: 73–121), and chloride (258 mEq/L; normal range: 110–250) excretion. His renin, aldosterone, and glomerular filtration rate all fell within the normal range. A heart and renal ultrasound were also performed, without detecting any alterations. Given the association of metabolic alkalosis with hypokalemia, hypomagnesemia, hypochloremia, hypocalciuria, and low to normal blood pressure levels, the patient was diagnosed with Gitelman syndrome. After a few days of intravenous therapy, his hydroelectric figures returned to normal, and subsequent correction of the electrocardiographic repolarization was observed, with no new arrhythmic events being detected during the rest of his hospital stay. At discharge, he was prescribed home treatment with spironolactone 100 mg/24 h, chronic oral supplementation with magnesium and potassium, as well as a diet rich in these electrolytes.</p><p id="par0020" class="elsevierStylePara elsevierViewall">Gitelman syndrome is not a benign pathology, as about half of patients with this condition have prolonged QT intervals,<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> with the consequent risk of potentially severe ventricular arrhythmias. Patients with this disease have myocardial perfusion alterations and microvascular dysfunction, both of which could act as precipitating factors for arrhythmic events in the context of chronic hypokalemia.<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> For this reason, once other secondary causes of hypokalemia, such as the use of laxatives or diuretics, have been ruled out, an early diagnostic suspicion of this disease based on clinical and biochemical parameters is very important.<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a></p><p id="par0025" class="elsevierStylePara elsevierViewall">We consider this case to be of interest due to the rarity of this condition, although with potentially serious consequences if it is not adequately managed and treated in a population that is usually young.</p><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Conflicts of interest</span><p id="par0030" class="elsevierStylePara elsevierViewall">The authors of this paper declare no conflicts of interest.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:2 [ 0 => array:2 [ "identificador" => "sec0005" "titulo" => "Conflicts of interest" ] 1 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as: Riaño Ondiviela A, Meseguer González D, Ruiz Arroyo JR. Arritmias ventriculares malignas en un paciente con síndrome de Gitelman. Med Clin (Barc). 2021;157:550.</p>" ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:4 [ 0 => array:3 [ "identificador" => "bib0005" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Clinical and diagnostic features of Bartter and Gitelman syndromes" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "P.R. Walsh" 1 => "Y. Tse" 2 => "E. Ashton" 3 => "D. Iancu" 4 => "L. Jenkins" 5 => "M. Bienias" ] ] ] ] ] "host" => array:1 [ 0 => array:1 [ "Revista" => array:5 [ "tituloSerie" => "Clin Kidney J" "fecha" => "2018" "volumen" => "11" "paginaInicial" => "302" "paginaFinal" => "309" ] ] ] ] ] ] 1 => array:3 [ "identificador" => "bib0010" "etiqueta" => "2" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Electrocardiogram with prolonged QT interval in Gitelman disease" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:6 [ 0 => "A. Bettinelli" 1 => "C. Tosetto" 2 => "G. Colussi" 3 => "G. Tommasini" 4 => "A. Edefonti" 5 => "M.G. Bianchetti" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1046/j.1523-1755.2002.00467.x" "Revista" => array:6 [ "tituloSerie" => "Kidney Int" "fecha" => "2002" "volumen" => "62" "paginaInicial" => "580" "paginaFinal" => "584" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/12110021" "web" => "Medline" ] ] ] ] ] ] ] ] 2 => array:3 [ "identificador" => "bib0015" "etiqueta" => "3" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Myocardial perfusion defects in Bartter and Gitelman syndromes" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:6 [ 0 => "R. Scognamiglio" 1 => "L.A. Caló" 2 => "C. Negut" 3 => "M. Coccato" 4 => "P. Mormino" 5 => "A.C. Pessina" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1111/j.1365-2362.2008.02034.x" "Revista" => array:6 [ "tituloSerie" => "Eur J Clin Invest" "fecha" => "2008" "volumen" => "38" "paginaInicial" => "888" "paginaFinal" => "895" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/19021712" "web" => "Medline" ] ] ] ] ] ] ] ] 3 => array:3 [ "identificador" => "bib0020" "etiqueta" => "4" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Gitelman syndrome: consensus and guidance from a Kidney Disease: improving Global Outcomes (KDIGO) controversies conference" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "A. Blanchard" 1 => "D. Bockenhauer" 2 => "D. Bolignano" 3 => "L.A. Calò" 4 => "E. Cosyns" 5 => "O. Devuyst" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1016/j.kint.2016.09.046" "Revista" => array:6 [ "tituloSerie" => "Kidney Int" "fecha" => "2017" "volumen" => "91" "paginaInicial" => "24" "paginaFinal" => "33" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/28003083" "web" => "Medline" ] ] ] ] ] ] ] ] ] ] ] ] ] "idiomaDefecto" => "en" "url" => "/23870206/0000015700000011/v1_202112030828/S2387020621006288/v1_202112030828/en/main.assets" "Apartado" => array:4 [ "identificador" => "43309" "tipo" => "SECCION" "en" => array:2 [ "titulo" => "Letters to the Editor" "idiomaDefecto" => true ] "idiomaDefecto" => "en" ] "PDF" => "https://static.elsevier.es/multimedia/23870206/0000015700000011/v1_202112030828/S2387020621006288/v1_202112030828/en/main.pdf?idApp=UINPBA00004N&text.app=https://www.elsevier.es/" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S2387020621006288?idApp=UINPBA00004N" ]
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