nach oben

Erschienen in:

01.12.2013 | Case Report

Fetal Long QT Syndrome Manifested as Atrioventricular Block and Ventricular Tachycardia: A Case Report and a Review of the Literature

verfasst von: Sanitra Anuwutnavin, Prapat Wanitpongpan, Paweena Chungsomprasong, Jarupim Soongswang, Nattinee Srisantiroj, Tuangsit Wataganara

Erschienen in: Pediatric Cardiology | Ausgabe 8/2013

Einloggen, um Zugang zu erhalten

Abstract

Fetal onset of congenital long QT syndrome (LQTS) is a rare manifestation, and prenatal diagnosis is difficult. This report describes a boy who presented with both atrioventricular (AV) block and ventricular tachycardia during the antenatal period. The early postnatal electrocardiogram showed prolongation of the QT interval and AV block, subsequently leading to a polymorphic ventricular tachycardia torsade de pointes. This unique feature of congenital LQTS has a poor outcome, but the boy was successfully treated with beta-blockers and implantation of an automated cardioverter-defibrillator. The intrauterine manifestation of fetal AV block and ventricular tachycardia should raise a high suspicion of congenital LQTS, and the strong association with a malignant clinical course should warrant special evaluation. The literature on the prenatal diagnosis, fetal therapy, and neonatal outcome of this condition also are reviewed.

Beinder E, Grancay T, Menendez T, Singer H, Hofbeck M (2001) Fetal sinus bradycardia and the long QT syndrome. Am J Obstet Gynecol 185:743–747CrossRefPubMed

Beinder E, Buheitel G, Hofbeck M (2003) Are some cases of sudden intrauterine unexplained death due to the long QT syndrome? Prenat Diagn 23:1097–1098CrossRefPubMed

Betrian Blasco P, Antunez Jimenez MI, Falcon Gonzalez LH, Suarez Cabrera P (2007) Neonatal life-threatening arrhythmia responding to lidocaine, a probable LQTS3. Int J Cardiol 117:e61–e63CrossRefPubMed

Blaufox AD (2012) Long QT syndrome in the very young: fetal life through infancy. Card Electrophysiol Clin 4:61–73CrossRef

Chang IK, Shyu MK, Lee CN, Kau ML, Ko YH, Chow SN et al (2002) Prenatal diagnosis and treatment of fetal long QT syndrome: a case report. Prenat Diagn 22:1209–1212CrossRefPubMed

Cuneo BF, Ovadia M, Strasburger JF, Zhao H, Petropulos T, Schneider J et al (2003) Prenatal diagnosis and in utero treatment of torsades de pointes associated with congenital long QT syndrome. Am J Cardiol 91:1395–1398CrossRefPubMed

Donofrio MT, Gullquist SD, O’Connell NG, Redwine FO (1999) Fetal presentation of congenital long QT syndrome. Pediatr Cardiol 20:441–444CrossRefPubMed

Duke C, Stuart G, Simpson JM (2005) Ventricular tachycardia secondary to prolongation of the QT interval in a fetus with autoimmune-mediated congenital complete heart block. Cardiol Young 15:319–321CrossRefPubMed

Fujimoto Y, Matsumoto T, Honda N, Tojo R, Furuya M, Kasai K et al (2009) Prenatal diagnosis of long QT syndrome by noninvasive fetal electrocardiography. J Obstet Gynaecol Res 35:555–561CrossRefPubMed

10.

Gorgels AP, Al Fadley F, Zaman L, Kantoch MJ, Al Halees Z (1998) The long QT syndrome with impaired atrioventricular conduction: a malignant variant in infants. J Cardiovasc Electrophysiol 9:1225–1232CrossRefPubMed

11.

Hamada H, Horigome H, Asaka M, Shigemitsu S, Mitsui T, Kubo T et al (1999) Prenatal diagnosis of long QT syndrome using fetal magnetocardiography. Prenat Diagn 19:677–680CrossRefPubMed

12.

Hofbeck M, Ulmer H, Beinder E, Sieber E, Singer H (1997) Prenatal findings in patients with prolonged QT interval in the neonatal period. Heart 77:198–204PubMedCentralCrossRefPubMed

13.

Hosono T, Kawamata K, Chiba Y, Kandori A, Tsukada K (2002) Prenatal diagnosis of long QT syndrome using magnetocardiography: a case report and review of the literature. Prenat Diagn 22:198–200CrossRefPubMed

14.

Komarlu R, Beerman L, Freeman D, Arora G (2012) Fetal and neonatal presentation of long QT syndrome. Pacing Clin Electrophysiol 35:e87–e90CrossRefPubMed

15.

Lin MT, Hsieh FJ, Shyu MK, Lee CN, Wang JK, Wu MH (2004) Postnatal outcome of fetal bradycardia without significant cardiac abnormalities. Am Heart J 147:540–544CrossRefPubMed

16.

Lupoglazoff JM, Denjoy I, Villain E, Fressart V, Simon F, Bozio A et al (2004) Long QT syndrome in neonates: conduction disorders associated with HERG mutations and sinus bradycardia with KCNQ1 mutations. J Am Coll Cardiol 43:826–830CrossRefPubMed

17.

Manning N, Anthony JP, Ostman-Smith I, Snyder CS, Burch M (2000) Prenatal diagnosis and successful preterm delivery of a fetus with long QT syndrome. BJOG 107:1049–1051CrossRefPubMed

18.

Medeiros-Domingo A, Iturralde-Torres P, Ackerman MJ (2007) Clinical and genetic characteristics of long QT syndrome. Rev Esp Cardiol 60:739–752CrossRefPubMed

19.

Menendez T, Achenbach S, Beinder E, Hofbeck M, Schmid O, Singer H et al (2000) Prenatal diagnosis of QT prolongation by magnetocardiography. Pacing Clin Electrophysiol 23:1305–1307CrossRefPubMed

20.

Miller TE, Estrella E, Myerburg RJ, Garcia de Viera J, Moreno N, Rusconi P et al (2004) Recurrent third-trimester fetal loss and maternal mosaicism for long-QT syndrome. Circulation 109:3029–3034CrossRefPubMed

21.

Miura M, Yamagishi H, Morikawa Y, Matsuoka R (2003) Congenital long QT syndrome and 2:1 atrioventricular block with a mutation of the SCN5A gene. Pediatr Cardiol 24:70–72CrossRefPubMed

22.

Ohkuchi A, Shiraishi H, Minakami H, Eguchi Y, Izumi A, Sato I (1999) Fetus with long QT syndrome manifested by tachyarrhythmia: a case report. Prenat Diagn 19:990–992CrossRefPubMed

23.

Sarkar S, Brumund M, Darling R, Snyder CS (2007) Survival of a newborn with 2:1 atrioventricular block, long QT Syndrome, and torsades de pointes. Ochsner J 7:181–184PubMedCentralPubMed

24.

Schmitz L, Burghoff M (2005) Images in cardiovascular medicine: magnetocardiography in a fetus with long-QT syndrome. Circulation 112:e68–e69CrossRefPubMed

25.

Schneider U, Haueisen J, Loeff M, Bondarenko N, Schleussner E (2005) Prenatal diagnosis of a long QT syndrome by fetal magnetocardiography in an unshielded bedside environment. Prenat Diagn 25:704–708CrossRefPubMed

26.

Schulze-Bahr E, Fenge H, Etzrodt D, Haverkamp W, Monnig G, Wedekind H et al (2004) Long QT syndrome and life-threatening arrhythmia in a newborn: molecular diagnosis and treatment response. Heart 90:13–16PubMedCentralCrossRefPubMed

27.

Schwartz PJ, Moss AJ, Vincent GM, Crampton RS (1993) Diagnostic criteria for the long QT syndrome: an update. Circulation 88:782–784CrossRefPubMed

28.

Schwartz PJ, Stramba-Badiale M, Segantini A, Austoni P, Bosi G, Giorgetti R et al (1998) Prolongation of the QT interval and the sudden infant death syndrome. N Engl J Med 338:1709–1714CrossRefPubMed

29.

Schwartz PJ, Stramba-Badiale M, Crotti L, Pedrazzini M, Besana A, Bosi G et al (2009) Prevalence of the congenital long-QT syndrome. Circulation 120:1761–1767PubMedCentralCrossRefPubMed

30.

Simpson JM (2006) Fetal arrhythmias. Ultrasound Obstet Gynecol 27:599–606CrossRefPubMed

31.

Simpson JM, Maxwell D, Rosenthal E, Gill H (2009) Fetal ventricular tachycardia secondary to long QT syndrome treated with maternal intravenous magnesium: case report and review of the literature. Ultrasound Obstet Gynecol 34:475–480CrossRefPubMed

32.

Takahashi K, Shiraishi H, Ohkuchi A, Matsubara S, Kuwata T, Yada Y et al (2009) Irregular peak-to-peak intervals between ascending aortic flows during fetal ventricular tachycardia in long QT syndrome. Ultrasound Obstet Gynecol 33:118–120CrossRefPubMed

33.

Tomek V, Skovranek J, Gebauer RA (2009) Prenatal diagnosis and management of fetal Long QT syndrome. Pediatr Cardiol 30:194–196CrossRefPubMed

34.

Tozzi RJ, Kurer CC, Kipel G (2003) Profound fetal bradycardia: a case report highlighting treatment options. J Perinatol 23:509–512CrossRefPubMed

35.

Trippel DL, Parsons MK, Gillette PC (1995) Infants with long-QT syndrome and 2:1 atrioventricular block. Am Heart J 130:1130–1134CrossRefPubMed

36.

Wu MH, Hsieh FC, Wang JK, Kau ML (1999) A variant of long QT syndrome manifested as fetal tachycardia and associated with ventricular septal defect. Heart 82:386–388PubMedCentralCrossRefPubMed

37.

Yao CT, Wang JN, Tsai YC, Lin CS, Wu JM (2002) Congenital long QT syndrome with functionally impaired atrioventricular conduction: successful treatment by mexiletine and propranolol. J Formos Med Assoc 101:291–293PubMed

Titel: Fetal Long QT Syndrome Manifested as Atrioventricular Block and Ventricular Tachycardia: A Case Report and a Review of the Literature
verfasst von: Sanitra Anuwutnavin
Prapat Wanitpongpan
Paweena Chungsomprasong
Jarupim Soongswang
Nattinee Srisantiroj
Tuangsit Wataganara
Publikationsdatum: 01.12.2013
Verlag: Springer US
Erschienen in: Pediatric Cardiology / Ausgabe 8/2013
Print ISSN: 0172-0643
Elektronische ISSN: 1432-1971
DOI: https://doi.org/10.1007/s00246-012-0507-1

Neu im Fachgebiet Kardiologie

Screening-Mammografie offenbart erhöhtes Herz-Kreislauf-Risiko

26.04.2024 Mammografie Nachrichten

Routinemäßige Mammografien helfen, Brustkrebs frühzeitig zu erkennen. Anhand der Röntgenuntersuchung lassen sich aber auch kardiovaskuläre Risikopatientinnen identifizieren. Als zuverlässiger Anhaltspunkt gilt die Verkalkung der Brustarterien.

Niedriger diastolischer Blutdruck erhöht Risiko für schwere kardiovaskuläre Komplikationen

25.04.2024 Hypotonie Nachrichten

Wenn unter einer medikamentösen Hochdrucktherapie der diastolische Blutdruck in den Keller geht, steigt das Risiko für schwere kardiovaskuläre Ereignisse: Darauf deutet eine Sekundäranalyse der SPRINT-Studie hin.

Therapiestart mit Blutdrucksenkern erhöht Frakturrisiko

25.04.2024 Hypertonie Nachrichten

Beginnen ältere Männer im Pflegeheim eine Antihypertensiva-Therapie, dann ist die Frakturrate in den folgenden 30 Tagen mehr als verdoppelt. Besonders häufig stürzen Demenzkranke und Männer, die erstmals Blutdrucksenker nehmen. Dafür spricht eine Analyse unter US-Veteranen.

Adipositas-Medikament auch gegen Schlafapnoe wirksam

24.04.2024 Adipositas Nachrichten

Der als Antidiabetikum sowie zum Gewichtsmanagement zugelassene Wirkstoff Tirzepatid hat in Studien bei adipösen Patienten auch schlafbezogene Atmungsstörungen deutlich reduziert, informiert der Hersteller in einer Vorab-Meldung zum Studienausgang.

Update Kardiologie

Bestellen Sie unseren Fach-Newsletter und bleiben Sie gut informiert.

Newsletter bestellen

Springer Medizin

Abstract

Bitte loggen Sie sich ein, um Zugang zu diesem Inhalt zu erhalten

Weitere Artikel der Ausgabe 8/2013

Effects of Race, Ethnicity, and Gender on Surgical Mortality in Hypoplastic Left Heart Syndrome

Elusive Pulmonary Venous Confluence-to-Hepatic Vein Connection Leading to Heart Failure

Massive Accumulation of Glycosaminoglycans in the Aortic Valve of a Patient With Hunter Syndrome During Enzyme Replacement Therapy

Pseudotumor Cerebri Associated With Modified Fontan Anatomy