Refractory supraventricular fetal tachycardia as a cause of non-immune hydrops: management conundrum.

Supraventricular tachyarrhythmia (SVT) is the most common form of fetal tachyarrhythmias. The presentation can vary from ill-defined, non-sustained episodes of tachyarrhythmia to frank non-immune hydrops. The standard of care is transplacental therapy by treating the mother with oral antiarrhythmic drugs, followed by direct fetal therapy in refractory cases. We report a case of primigravida in her late 20s, who presented at 28.1 weeks of gestation with fetal hydrops and SVT. She was initially managed with oral digoxin and flecainide, but due to worsening hydrops, risk of fetal demise and extreme prematurity, further management by direct fetal therapy was given in terms of intramuscular digoxin and intraperitoneal flecainide. Following which, the fetus had a favourable outcome. This case highlights the possible role of direct fetal therapy in refractory cases of SVT.

Prenatally Diagnosed Large Lung Lesions: Timing of Resection and Perinatal Outcomes.

INTRODUCTION: Fetuses with large lung lesions including congenital cystic adenomatoid malformations (CCAMs) are at risk for cardiopulmonary compromise. Prenatal maternal betamethasone and cyst drainage for micro- and macrocystic lesions respectively have improved outcomes yet some lesions remain large and require resection before birth (open fetal surgery, OFS), at delivery via an Ex Utero Intrapartum Treatment (EXIT), or immediately post cesarean section (section-to-resection, STR). We sought to compare prenatal characteristics and outcomes in fetuses undergoing OFS, EXIT, or STR to inform decision-making and prenatal counseling. METHODS: A single institution retrospective review was conducted evaluating patients undergoing OFS, EXIT, or STR for prenatally diagnosed lung lesions from 2000 to 2021. Specimens were reviewed by an anatomic pathologist. Lesions were divided into "CCAMs" (the largest pathology group) and "all lung lesions" since pathologic diagnosis is not possible during prenatal evaluation when care decisions are made. Prenatal variables included initial, greatest, and final CCAM volume-ratio (CVR), betamethasone use/frequency, cyst drainage, and the presence of hydrops. Outcomes included survival, ECMO utilization, NICU length of stay (LOS), postnatal nitric oxide use, and ventilator days. RESULTS: Sixty-nine percent (59 of 85 patients) of lung lesions undergoing resection were CCAMs. Among patients with pathologic diagnosis of CCAM, the initial, largest, and final CVRs were greatest in OFS followed by EXIT and STR patients. Similarly, the incidence of hydrops was significantly greater and the rate of hydrops resolution was lower in the OFS group. Although the rate of cyst drainage did not differ between groups, maternal betamethasone use varied significantly (OFS 60.0%, EXIT 100.0%, STR 74.3%; p = 0.0378). Notably, all OFS took place prior to 2014. There was no difference in survival, ventilator days, nitric oxide, NICU LOS, or ECMO between groups. In multiple variable logistic modeling, determinants of survival to NICU discharge among patients undergoing resection with a pathologic diagnosis of CCAM included initial CVR <3.5 and need for <3 maternal betamethasone doses. CONCLUSION: For CCAMs that remain large despite maternal betamethasone or cyst drainage, surgical resection via OFS, EXIT, or STR are viable options with favorable and comparable survival between groups. In the modern era there has been a shift from OFS and EXIT procedures to STR for fetuses with persistently large lung lesions. This shift has been fueled by the increased use of maternal betamethasone and introduction of a Special Delivery Unit during the study period and the appreciation of similar fetal and neonatal outcomes for STR vs. EXIT and OFS with reduced maternal morbidity associated with a STR. Accordingly, efforts to optimize multidisciplinary perinatal care for fetuses with large lung lesions are important to inform patient selection criteria and promote STR as the preferred surgical approach in the modern era. LEVEL OF EVIDENCE: Level IV.

Transplacental Therapeutic Drug Monitoring in Pregnant Women with Fetal Tachyarrhythmia Using HPLC-MS/MS.

Fetal arrhythmia develops in 0.1-5% of pregnancies and may cause fetal heart failure and fetal hydrops, thus increasing fetal, neonatal, and infant mortality. The timely initiation of transplacental antiarrhythmic therapy (ART) promotes the conversion of fetal tachycardia to sinus rhythm and the regression of the concomitant non-immune fetal hydrops. The optimal treatment regimen search for the fetus with tachyarrhythmia is still of high value. Polymorphisms of these genes determines the individual features of the drug pharmacokinetics. The aim of this study was to study the pharmacokinetics of transplacental anti-arrhythmic drugs in the fetal therapy of arrhythmias using HPLC-MS/MS, as well as to assess the effect of the multidrug-resistance gene ABCB1 3435C > T polymorphism on the efficacy and maternal/fetal complications of digoxin treatment. The predisposition to a decrease in the bioavailability of the digoxin in patients with a homozygous variant of the CC polymorphism showed a probable association with the development of ART side effects. A pronounced decrease in heart rate in women with the 3435TT allele of the ABCB1 gene was found. The homozygous TT variant in the fetus showed a probable association with an earlier response to ART and rhythm disruptions on the digoxin dosage reduction. high-performance liquid chromatography with tandem mass spectrometry (HPLC-MS/MS) methods for digoxin and sotalol therapeutic drug monitoring in blood plasma, amniotic fluid, and urine were developed. The digoxin and sotalol concentrations were determined in the plasma blood, urine, and amniotic fluid of 30 pregnant women at four time points (from the beginning of the transplacental antiarrhythmic therapy to delivery) and the plasma cord blood of 30 newborns. A high degree of correlation between the level of digoxin and sotalol in maternal and cord blood was found. The ratio of digoxin and sotalol in cord blood to maternal blood was 0.35 (0.27 and 0.46) and 1.0 (0.97 and 1.07), accordingly. The digoxin concentration in the blood of the fetus at the moment of the first rhythm recovery episode, 0.58 (0.46, 0.8) ng/mL, was below the therapeutic interval. This confirms the almost complete transplacental transfer of sotalol and the significant limitation in the case of digoxin. Previously, ABCB1/P-glycoprotein had been shown to limit fetal exposure to drugs. Further studies (including multicenter ones) to clarify the genetic features of the transplacental pharmacokinetics of antiarrhythmic drugs are needed.

Postnatal Outcomes in Infants With a History of Fetal Supraventricular Tachycardia.

BACKGROUND: Fetal supraventricular tachycardia (SVT) is rare and proposed predictors of postnatal outcomes in fetal SVT have not been validated. Valid predictors can guide postnatal management. OBJECTIVES: The authors correlated fetal characteristics to the incidence of postnatal SVT and compared SVT outcomes in infants with and without a history of fetal SVT. METHODS: Mother-fetus dyads with fetal SVT and a structurally normal heart were described and compared with a second cohort of infants with a postnatal diagnosis of SVT. RESULTS: SVT was observed in 78 fetuses and 76 survived to delivery. Maternally administered transplacental antiarrhythmics were used in 49 mother-fetus dyads. Rhythm control was achieved in 37 of 49 (76%). Among fetuses with intermittent SVT, there was no ventricular dysfunction or hydrops. Postnatal SVT occurred in one-half of infants (37 of 76), and 94% presented within the first 2 days of life. The following fetal characteristics were associated with postnatal SVT on univariable analysis: sustained SVT (87% vs 56%), ventricular dysfunction (41% vs 15%), lack of conversion to sinus rhythm (49% vs 10%), and earlier gestational age at delivery (37.6 weeks vs 38.9 weeks; P ≤ 0.01 for each comparison). Compared with infants with a postnatal diagnosis of SVT, infants with a fetal diagnosis presented earlier (median age 0 days vs 17 days; P < 0.01) and had a lower incidence ventricular dysfunction at presentation (5% vs 42%; P < 0.01). CONCLUSIONS: One-half of infants with fetal SVT had postnatal SVT, nearly all within 2 days of life. These data and predictors of postnatal SVT may influence parental counseling and postnatal clinical decision-making.

Treatment of Severe Fetal Ebstein's Anomaly with Prenatal Nonsteroidal Anti-Inflammatory Therapy.

INTRODUCTION: Prenatally diagnosed Ebstein's anomaly with tricuspid valve dysplasia (EA/TVD) is a rare and high-risk congenital heart malformation with limited effective treatments. We report a case of severe fetal EA with hydrops treated with modest doses of nonsteroidal anti-inflammatory drug (NSAID) therapy, resulting in reversal of hydrops and a favorable fetal outcome. CASE PRESENTATION: Fetal heart defects included an inferiorly displaced tricuspid valve, severe tricuspid regurgitation, significantly dilated right atrium, and hypoplastic pulmonary valve with moderate regurgitation resulting in a circular shunt across the ductus arteriosus. Maternal indomethacin therapy was initiated at 31+5 weeks gestation due to the development of fetal hydrops as demonstrated by the presence of a pericardial effusion and ascites. Indomethacin therapy resulted in the desired restriction of the ductus arteriosus and resolution of fetal hydrops. Maternal therapy was transitioned to ibuprofen and serial fetal echocardiograms ensured continued ductal restriction. Delivery occurred via cesarean at 36+3 weeks. The neonate did not require immediate cardiac surgical intervention and was discharged home with close follow-up. DISCUSSION/CONCLUSION: A lower dose of prenatal NSAID therapy effected successful ductal restriction and hemodynamic mitigation of the circular shunt, resulting in reversal of hydrops and avoidance of postnatal cardiac surgical intervention.

Congenital syphilis with hydrops fetalis: report of four cases in a general referral hospital in Bogota, Colombia between 2016- 2020 / Sífilis congénita con hídrops fetal: reporte de cuatro casos en un hospital general de referencia en Bogotá, Colombia entre 2016- 2020

Objective: To report four cases of hydrops fetalis secondary to congenital syphilis and carry out a review of the literature to answer the question, What is the antibiotic regimen used in cases of gestational syphilis with hydrops fetalis as a complication? Materials and Methods: Four cases of congenital syphilis with hydrops fetalis are presented. Maternal age ranged between 17 and 28 years, gestational age at the time of diagnosis varied between 25 and 30 weeks, and two of the mothers had not initiated prenatal care at that time. Treatment with crystalline penicillin for gestational syphilis was given immediately 6 to 12 weeks before delivery in three cases and partners were prescribed treatment with benzathine penicillin. As for the neonates, two had no active infection or sequelae and one of them was considered to have congenital syphilis based on non-treponemal test titers. In one case, the patient was unable to receive syphilis treatment before delivery and her newborn had signs of active infection. A review of the literature was conducted in the Medline, LILACS and Google Scholar databases using the search terms "hydrops fetalis," "Lues", "syphilis ­ prenatal diagnosis - ultrasound - penicillin ­ treatment". The search included case reports and case series or cohorts of newborns with gestational syphilis and hydrops fetalis. Information regarding treatment in the mothers and in the newborns was extracted. Results: Overall, 119 articles were identified. Of these, 13 met the inclusion criteria, three were discarded because the full text could not be accessed. Ten studies with a total of 16 reported cases of hydrops fetalis secondary to congenital infection were reported. Of these, three presented with severe fetal anemia and required intrauterine transfusion; 5 cases received intrauterine penicillin treatment. In four cases the mother received weekly intramuscular injections of benzathine penicillin for 3 weeks, one received additional intravenous crystalline penicillin for 13 days, while another one received intravenous crystalline penicillin for 14 days. Treatment during gestation was not given in a total of 11 cases; and 6 of the 16 cases (37.5%) resulted in perinatal death. Conclusion: Delays in prenatal care and late diagnosis and treatment of gestational syphilis are important causes of persistent congenital syphilis. Randomized studies are required to identify the best treatment in fetuses with congenital syphilis 30 days before delivery and in fetuses with systemic compromise during the second half of gestation.

Objetivo: realizar un reporte de 4 casos de hídrops fetal secundario a sífilis congénita y hacer una revisión de la literatura para responder la siguiente pregunta: ¿cuál es el esquema antibiótico utilizado en los casos de sífilis gestacional complicada con hídrops fetal? Materiales y métodos: se presentan 4 casos de sífilis congénita con hídrops fetal. La edad materna varió entre 17 y 28 años, la edad gestacional al momento del diagnóstico estuvo entre 25 y 30 semanas, dos de ellas no habían iniciado control prenatal en ese momento. En tres casos se dio tratamiento para sífilis gestacional inmediatamente con penicilina cristalina entre 6 y 12 semanas antes del parto y se formuló tratamiento a la pareja con penicilina benzatínica. Respecto a los recién nacidos, dos de ellos no tenían infección activa o secuelas, se consideró que uno de ellos tenía sífilis congénita por títulos de prueba no treponémica. En uno de los casos, la paciente no alcanza a recibir tratamiento para la sífilis gestacional antes del parto, este recién nacido tenía signos de infección activa. Se hizo una revisión de la literatura en las bases de datos Medline, LILACS y google scholar; los términos de búsqueda fueron los siguientes: "hídrops fetal", "lues", "syphilis ­ prenatal diagnosis- ultrasound - penicilina ­ treatment". Se buscaron reportes y series de casos o cohortes de recién nacidos con sífilis gestacional con hídrops fetalis. Se extrajo información sobre la madre y el recién nacido respecto al tratamiento. Resultados: se identificaron 119 artículos, de estos 13 cumplieron con los criterios de inclusión, tres fueron descartados por no tener acceso al texto completo. Se incluyeron diez estudios de un total de 16 casos reportados con diagnóstico prenatal de hídrops fetal secundarios a infección congénita. De ellos, tres presentaron anemia fetal severa y requirieron transfusión intrauterina; 5 casos recibieron tratamiento intrauterino con penicilina. En cuatro casos la madre recibió penicilina benzatínica intramuscular por 3 semanas, uno recibió además penicilina cristalina endovenosa por 13 días, otro recibió penicilina cristalina endovenosa por 14 días. Un total de 11 casos no recibieron tratamiento durante la gestación; 6 de los 16 casos (37,5%) presentaron muerte perinatal. Conclusión: el retraso en acudir al control prenatal y la tardanza del diagnóstico y tratamiento de la sífilis gestacional son causas importantes de la persistencia de la sífilis congénita. Se requieren estudios aleatorizados para determinar el mejor tratamiento del feto con sífilis congénita en los 30 días previos al parto y del feto con compromiso sistémico durante la segunda mitad de la gestación.

Complete resolution of arrhythmia-induced hydrops fetalis in utero.

A 21-year-old G3P2011 Caucasian woman at 27 weeks' gestation presented with fetal tachyarrhythmia between 240 and 270 beats per minute. Fetal supraventricular tachycardia, abdominal ascites, pleural effusion and pericardial effusion indicated hydrops fetalis. Management with digoxin and flecainide converted the fetus to sinus rhythm and resolved the ascites and pleural effusion within 4 days of treatment. Flecainide was discontinued at 31 weeks' gestation due to elevated liver enzymes. Intrahepatic cholestasis was treated with ursodiol. Caesarean section was performed at 37 weeks' gestation. Neonatal echocardiogram revealed a bicuspid aortic valve with mild regurgitation and a patent foramen ovale, and the infant showed no subsequent evidence of tachyarrhythmia or hydrops after delivery. Treatment of hydrops fetalis in the antenatal period is complex, and early diagnosis and treatment can quickly resolve supraventricular tachycardia-induced hydrops fetalis.

Resolution of Fetal Hydrops Dependent on Sustained Fetal Supraventricular Tachycardia after Digoxin Therapy.

We present a special case of fetal supraventricular tachycardia detected at 34 weeks gestation. Fetal hydrops was noted on ultrasound upon admission. Normal fetal heart rate was maintained for three weeks by maternal administration of digoxin. A live infant was delivered via caesarian section at 37 weeks gestation. This clinical case demonstrated that pharmacological treatment can be effective and helps to prolong pregnancy safely.

Fetal arrhythmias: diagnosis and treatment.

Fetal arrhythmias are common, and they may resolve spontaneously in majority of the cases. Sustained fetal arrhythmias associated with major structural heart disorders, hydrops fetalis, and fetal heart failure warrant intrauterine pharmaceutical conversion of heart rhythm or early pacemaker implant in order to avoid fetal demise. Fetal atrial flutter (AF) and supraventricular tachycardia (SVT) resemble in terms of the effects of intrauterine therapies. Digoxin is more suitable for rhythm conversion of fetal AF and SVT in fetuses free of hydrops fetalis, while sotalol shows better effects for those with hydrops fetalis. In fetal cases of atrioventricular blocks, an etiological treatment for the maternal antibody exposure by steroids could be an alternative remedy. In this article, the clinical diagnosis and treatment of fetal arrhythmias are presented, and advantages and disadvantages of antiarrhythmic agents for fetal arrhythmias are compared.

Sotalol as an effective adjunct therapy in the management of supraventricular tachycardia induced fetal hydrops fetalis.

Sustained fetal supraventricular tachycardia (SVT) complicated by hydrops fetalis carries a significant risk of morbidity and mortality. While there is no clear consensus on first- and second-line therapy options for the management of fetal SVT with or without hydrops fetalis, there exists significant nonrandomized experience with a number of antiarrhythmic agents that has founded the basis for management. Furthermore, recently published meta-analyses and ongoing multicenter prospective studies have aimed to bridge the gap in the literature. We report two cases of sustained fetal SVT with severe secondary hydrops fetalis managed successfully with flecainide-sotalol combination therapy in one case and sotalol-digoxin combination therapy in the second and review the literature for the management of fetal SVT.

Maternal steroid therapy for fetuses with immune-mediated complete atrioventricular block: a systematic review and meta-analysis.

INTRODUCTION: To explore the effect of maternal fluorinated steroid therapy on fetuses affected by immune-mediated complete atrio-ventricular block (CAVB) in utero. MATERIAL AND METHODS: Pubmed, Embase, Cinahl, and ClinicalTrials.gov databases were searched. Only studies reporting the outcome of fetuses with immune CAVB diagnosed on prenatal ultrasound without any cardiac malformations and treated with fluorinated steroids compared to those not treated were included. The primary outcome observed was the regression of CAVB; secondary outcomes were need for pacemaker insertion, overall mortality, defined as the occurrence of either intrauterine (IUD) or neonatal (NND) death, IUD, NND, termination of pregnancy (TOP). Furthermore, we assessed the occurrence of all these outcomes in hydropic fetuses compared to those without hydrops at diagnosis. Meta-analyses of proportions using random effect model and meta-analyses using individual data random-effect logistic regression were used to combine data. RESULTS: Eight studies (162 fetuses) were included. The rate of regression was 3.0% (95%CI 0.2-9.1) in fetuses treated and 4.3% (95%CI 0.4-11.8) in those not treated, with no difference between the two groups (odds ratio (OR): 0.9, 95%CI 0.1-15.1). Pacemaker at birth was required in 71.5% (95%CI 56.0-84.7) of fetuses-treated and 57.8% (95%CI 40.3-74.3) of those not treated (OR: 9, 95%CI 0.4-3.4). There was no difference in the overall mortality rate (OR: 0.5, 95%CI 0.9-2.7) between the two groups; in hydropic fetuses, mortality occurred in 76.2% (95%CI 48.0-95.5) of the treated and in 23.8% (95%CI 1.2-62.3) of the untreated group, while in those without hydrops the corresponding figures were 8.9% (95%CI 2.0-20.3) and 12% (95%CI 8.7-42.2), respectively. Improvement or resolution of hydrops during pregnancy occurred in 76.2% (95%CI 48.0-95.5) of cases treated and in 23.3% (95%CI 1.2-62.3) of those nontreated with fluorinated steroids. CONCLUSIONS: The findings from this systematic review do not suggest a potential positive contribution of antenatal steroid therapy in improving the outcome of fetuses with immune CAVB.

Resolution of hydrops fetalis caused by atrioventricular block: good postnatal evolution with terbutaline treatment.

INTRODUCTION: Complete atrioventricular block (CAVB) is rarely seen, as it occurs in only 1:11 000 to 1:20 000 newborns. There is a serious risk of mortality in CAVB, mainly in those cases associated with hydrops, fetal cardiac frequency ≤ 55 beats/minute, and premature delivery. CASE REPORT: Case of complete atrioventricular block with a poor prognosis (hydrops fetalis and foetal cardiac frequency < 5 beats/minute) caused by anti-La and anti-Ro antibodies. Intrauterine symptoms improved after treatment with terbutaline, permit- ting foetal viability and successful postnatal treatment with a cardiac pacemaker. DISCUSSION: In case of complete atrioventricular block of cause autoimmune with poor prognosis should be treated with positive inotropic drugs, anticholinergics or b-mimetic in the attempt to maintain adequate ventricular frequency, and thus prevent hydrops fetalis from occurring.

Diagnosis and treatment of foetal heart failure: foetal echocardiography and foetal hydrops.

Foetal echocardiography has progressed to be able to diagnose many forms of CHD and to assess the prognosis of cardiac lesions based on their anatomy and presentation in utero. This article outlines a straightforward method for the rapid evaluation of foetus that may have congestive heart failure with or without hydrops and for the differentiation of the pre-hydropic state from normal. The presence of signs of foetal heart failure, such as cardiomegaly or valvular regurgitation, gives clues to the aetiology of hydrops. The assessment of the prognosis of hydrops foetalis can be difficult but can be aided by the use of the cardiovascular profile score. Once identified, the neurohumoral effects of foetal heart failure can be ameliorated using transplacental digoxin if the hydrops has not progressed.

Sobrecarga de ferro em adolescente com xerocitose: a importância da ressonância nuclear magnética / Iron overload in a teenager with xerocytosis: the importance of nuclear magnetic resonance imaging

Relatar um caso de sobrecarga de ferro secundária à xerocitose, uma doença rara, em uma adolescente, diagnosticada por meio de ressonância magnética em T2*. Relatamos o caso de uma paciente sintomática com xerocitose, nível de ferritina de 350ng/mL e sobrecarga de ferro cardíaca significativa. Ela foi diagnosticada por ressonância magnética em T2* e recebeu terapia de quelação. Análise por ectacitometria confirmou o diagnóstico de xerocitose hereditária. Na sequência, a ressonância magnética em T2* demonstrou resolução completa da sobrecarga de ferro em vários órgãos e novo ecocardiograma revelou resolução completa das alterações cardíacas anteriores. A paciente permanece em terapia de quelação. Xerocitose é uma desordem genética autossômica dominante rara, caracterizada por estomatocitose desidratada. O paciente pode apresentar fadiga intensa e sobrecarga de ferro. Sugerimos o uso regular de ressonância magnética em T2* para o diagnóstico e controle da resposta à quelação de ferro em xerocitose e acreditamos que o exame pode ser útil também em outras anemias hemolíticas que necessitam de transfusões.

To report a case of iron overload secondary to xerocytosis, a rare disease in a teenager, diagnosed, by T2* magnetic resonance imaging. We report the case of a symptomatic patient with xerocytosis, a ferritin level of 350ng/mL and a significant cardiac iron overload. She was diagnosed by T2* magnetic resonance imaging and received chelation therapy Ektacytometric analysis confirmed the diagnosis of hereditary xerocytosis. Subsequent T2* magnetic resonance imaging demonstrated complete resolution of the iron overload in various organs, as a new echocardiography revealed a complete resolution of previous cardiac alterations. The patient remains in chelation therapy. Xerocytosis is a rare autosomal dominant genetic disorder characterized by dehydrated stomatocytosis. The patient may present with intense fatigue and iron overload. We suggest the regular use of T2* magnetic resonance imaging for the diagnosis and control of the response to iron chelation in xerocytosis, and we believe it can be used also in other hemolytic anemia requiring transfusions.

Non-immune hydrops fetalis caused by herpes simplex virus type 2 in the setting of recurrent maternal infection.

We report a case of non-immune hydrops fetalis (NIHF) caused by herpes simplex virus type 2 (HSV-2) in an infant whose mother had recurrent HSV-2 infection. In spite of prematurity, severe disseminated infection and hydrops, the infant survived and was neurologically intact. HSV-2-induced NIHF is extremely rare, particularly in the setting of recurrent maternal infection, and this case is, to our knowledge, the first report of a surviving infant. HSV-2 should be considered in the differential diagnosis of NIHF and early initiation of empiric acyclovir therapy is recommended in this setting, pending the results of virologic diagnostic tests.

Iron overload in a teenager with xerocytosis: the importance of nuclear magnetic resonance imaging.

To report a case of iron overload secondary to xerocytosis, a rare disease in a teenager, diagnosed, by T2* magnetic resonance imaging. We report the case of a symptomatic patient with xerocytosis, a ferritin level of 350ng/mL and a significant cardiac iron overload. She was diagnosed by T2* magnetic resonance imaging and received chelation therapy Ektacytometric analysis confirmed the diagnosis of hereditary xerocytosis. Subsequent T2* magnetic resonance imaging demonstrated complete resolution of the iron overload in various organs, as a new echocardiography revealed a complete resolution of previous cardiac alterations. The patient remains in chelation therapy. Xerocytosis is a rare autosomal dominant genetic disorder characterized by dehydrated stomatocytosis. The patient may present with intense fatigue and iron overload. We suggest the regular use of T2* magnetic resonance imaging for the diagnosis and control of the response to iron chelation in xerocytosis, and we believe it can be used also in other hemolytic anemia requiring transfusions.