A Case of Uncorrected Tetralogy of Fallot Undiagnosed Until Adulthood and Presenting With Polycythemia

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Abstract

Congenital heart defects with right-to-left shunt are one of the hypoxia-related causes of acquired secondary polycythemia (SP). Tetralogy of Fallot (ToF) is the most common congenital cyanotic heart disease in children. Cases of uncorrected ToF in adult ages are rare. This paper reports a woman detected with elevated hemoglobin level during routine tests performed for infertility therapy and subsequently diagnosed SP with related ToF.

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Long-term survival in patients with repair of tetralogy of Fallot: 36-year follow-up of 490 survivors of the first year after surgical repair.

Georg Nollert, Teddy Fischlein, Stefan Bouterwek … (1997)

We sought to analyze risk factors for long-term survival (up to 36 years) after surgical repair of tetralogy of Fallot (TOF). Survival after repair is excellent, but data >20 years are rare. From 1958 to 1977, 658 patients underwent correction of TOF at our institution and were analyzed for survival. Of this patient group (age 12.2 +/- 8.6 years [mean +/- SD], range 2 to 67), 39.7% had a previous palliation. Operative (n = 139) and 1-year (n = 29) deaths were excluded for long-term calculations, resulting in a study group of 490 patients. Actuarial 10-, 20-, 30- and 36-year survival rates were 97%, 94%, 89% and 85%, respectively. Mortality increased 25 years postoperatively from 0.24%/year to 0.94%/year (p = 0.003). The most common cause of death was sudden death (n = 13), followed by congestive heart failure (n = 6). Multivariate correlates of impaired long-term survival were date of operation (before 1970, p = 0.0104), preoperative polycythemia (p = 0.0487) and use of a right ventricular (RV) outflow patch (p = 0.0079). Postoperative systolic RV/left ventricular pressure ratio and age showed no influence. Patients without preoperative polycythemia and an RV outflow patch (n = 164) had a 36-year actuarial survival rate of 96% and normal life expectancy. Cyanosis, operative experience of the surgeon and an RV outflow tract patch influence long-term outcome after repair of TOF in older children. Early repair by experienced surgeons to avoid polycythemia and excessive RV hypertrophy is supported by this study. However, mortality risk increases 25 years postoperatively, and thus heart monitoring should be intensified.

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Uncorrected tetralogy of Fallot in an 86-year-old patient.

Alvaro Alonso-Garbayo, Brian C Downey, Jeffrey T Kuvin (2016)

This report describes the presentation and evaluation of an elderly man with uncorrected tetralogy of Fallot. The patient had remained fairly asymptomatic for much of his life. He presented to the hospital at age 86 with new-onset atrial fibrillation with rapid ventricular response and a non-ST-segment elevation myocardial infarction. Transthoracic and transesophageal echocardiography revealed infundibular pulmonic stenosis with a ventricular septal defect, overriding aorta, and right ventricular hypertrophy, findings consistent with unrepaired tetralogy of Fallot. Severe right ventricular pressure overload was also present. Coronary angiography revealed nonobstructive coronary artery disease. It was felt that the rapid atrial fibrillation resulted in right ventricular subendocardial ischemia that improved following restoration of sinus rhythm. After a systematic literature search, the authors believe this case represents the oldest reported patient with the diagnosis of uncorrected tetralogy of Fallot and serves as an example of a well-balanced congenital shunt.

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Red cell mass and plasma volume measurements in polycythemia: evaluation of performance and practical utility.

Ayalew Tefferi, V F Fairbanks, Shireen Sirhan (2005)

Despite the absence of any systematic evidence for diagnostic utility, red cell mass (RCM) measurement has been endorsed as a major diagnostic criterion for polycythemia vera (PV) based on a set of eligibility criteria for a clinical trial formulated by an International PV Study Group in 1967. A retrospective analysis was performed on a consecutive series of 105 patients who underwent blood volume measurements for evaluation of polycythemia. In a previous study, the authors had systematically compared RCM measurement by 51Cr-labeled erythrocytes and 125I-labeled human serum albumin and demonstrated equivalence between the two methods. In the current study, they used the latter method and applied the International Committee for Standardization in Haematology recommendations for result interpretation in order to evaluate test performance and practical utility. RCM exceeded the 98-99% limits of the reference range in 76%, 20%, 22%, and 57% of patients with PV (n = 25), secondary polycythemia (n = 35), spurious or apparent polycythemia (n = 38), and essential thrombocythemia (n = 7), respectively. Decreased plasma volume was rarely seen in any of the disease categories. In all instances of PV, the diagnosis was readily apparent, based on alternative clinical and laboratory tests, and did not require the additional information from blood volume measurement. Furthermore, alternative methods of PV diagnosis, based on disease-specific biological markers as well as bone marrow histology, are now available. The continued use of RCM and plasma volume measurements for the diagnosis of PV is no longer warranted.

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Author and article information

Journal

Journal ID (nlm-ta): Cardiol Res

Journal ID (iso-abbrev): Cardiol Res

Journal ID (publisher-id): Elmer Press

Title: Cardiology Research

Publisher: Elmer Press

ISSN (Print): 1923-2829

ISSN (Electronic): 1923-2837

Publication date (Print): December 2014

Publication date (Electronic): 04 December 2014

Volume: 5

Issue: 6

Pages: 198-200

Affiliations

[a ]Department of Emergency Medicine, Faculty of Medicine, Dicle University, Diyarbakir, Turkey

[b ]Department of Endocrinology, Diyarbakir Education and Research Hospital, Diyarbakir, Turkey

[c ]Department of Cardiology, Faculty of Medicine, Dicle University, Diyarbakir, Turkey

Author notes

[d ]Corresponding Author: Ercan Gunduz, Department of Emergency Medicine, Dicle University, 21280-Diyarbakir, Turkey. Email: drercangunduz@ 123456hotmail.com

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DOI: 10.14740/cr374e

PMC ID: 5358271

PubMed ID: 28352455

SO-VID: 56a2b9e3-cc42-4a7e-aca0-d8e61e5fb409

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This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

A Case of Uncorrected Tetralogy of Fallot Undiagnosed Until Adulthood and Presenting With Polycythemia

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Abstract

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Most cited references 8

Long-term survival in patients with repair of tetralogy of Fallot: 36-year follow-up of 490 survivors of the first year after surgical repair.

Uncorrected tetralogy of Fallot in an 86-year-old patient.

Red cell mass and plasma volume measurements in polycythemia: evaluation of performance and practical utility.

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