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CLINICAL RESEARCH: CONGENITAL HEART DISEASE

Structural Abnormalities of the Pulmonary Trunk in Tetralogy of Fallot and Potential Clinical Implications

A Morphological Study

Adult Congenital Heart Centre/Centre for Pulmonary Hypertension and Unit of Cardiac Morphology, Royal Brompton Hospital and the National Heart and Lung Institute, Imperial College London, London, United Kingdom

Manuscript received April 6, 2009; revised manuscript received May 19, 2009, accepted June 1, 2009.

^_* Reprint requests and correspondence: Prof. Siew Yen Ho, Cardiac Morphology, National Heart and Lung Institute, Imperial College London and Royal Brompton Hospital, Dovehouse Street, London SW3 6LY, United Kingdom (Email: yen.ho{at}imperial.ac.uk).

Objectives: The purpose of this study was to determine whether intrinsic histological abnormalities of the pulmonary trunk (PT) are present from birth and interact with palliative surgery and/or repair.

Background: Little is known about PT histology in patients with tetralogy of Fallot (TOF), especially in the era of surgical intervention in childhood.

Methods: We studied 39 formalin-fixed necropsy heart specimens with TOF and compared them with 17 normal control heart specimens. Sections of the PT and aorta were studied by light microscopy using various stains; histological findings were graded according to severity.

Results: Among the TOF group (1 fetus, 11 infants, 14 children, and 13 adults), 11 patients had undergone palliative and 10 patients had undergone reparative surgery at a median age of 8 years (range 2.5 to 18 years). Histological changes of grade 2 or higher were present in 59% (medionecrosis), 36% (fibrosis), 56% (cystlike formation), and 56% (abnormal elastic tissue configuration) of TOF patients. Total histology grading scores were higher in TOF hearts (median 6, range 1 to 9) compared with controls (median 1, range 0 to 6; p < 0.0001). Histological abnormalities were present among infants (median score 3.5, range 1 to 9) and after palliative surgery (median score 5, range 2 to 9) or repair (median score 7.5, range 4 to 9).

Conclusions: Marked histological abnormalities in the PT of hearts with TOF exist compared with controls. These changes were present from infancy and among patients who had undergone palliative or reparative surgery, although operations in this cohort were performed late. Our data suggest that structural abnormalities of the PT, similar to these recently shown in the aorta, are intrinsic.

Key Words: arteries • structure • tetralogy of Fallot • tissue

Abbreviations and Acronyms   Ao/LV index = aortic circumference indexed to the length of the left ventricle   CLF = cystlike formation   CMN = cystic medial necrosis   ETC = elastic tissue configuration   HGS = histological grading score(s)   PA = pulmonary atresia   PR = pulmonary regurgitation   PS = pulmonary valve stenosis   PT = pulmonary trunk   PT/Ao MT ratio = pulmonary trunk-to-aortic media thickness ratio   PT/LV index = pulmonary trunk circumference indexed to the length of the left ventricle   RV = right ventricular   TOF = tetralogy of Fallot

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J. Am. Coll. Cardiol. 2009 54: A32. [Full Text] [PDF]