This Article Extract Full Text (PDF) Data Supplement Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Request Permissions Citing Articles Citing Articles via Google Scholar Google Scholar Articles by Bertram, H. Articles by Yelbuz, T. M. Search for Related Content PubMed PubMed Citation Articles by Bertram, H. Articles by Yelbuz, T. M. Related Collections Pulmonary circulation and disease Other diagnostic testing Pediatric and congenital heart disease, including cardiovascular surgery

(Circulation. 2008;118:e665-e666.)
© 2008 American Heart Association, Inc.

Images in Cardiovascular Medicine

Rapid Regression of Classic Clinical Signs of Cyanosis Accompanied by Disappearing Major Aortopulmonary Collaterals After Surgical Palliation Only in a 6-Year-Old Girl With Tetralogy of Fallot

Harald Bertram, MD; Thomas Jack, MD; Dietmar Schranz, MD, PhD; Thomas Breymann, MD; Armin Wessel, MD, PhD; T. Mesud Yelbuz, MD, PhD

From the Department of Pediatric Cardiology and Intensive Care Medicine, Hannover Medical School, Hannover (H.B., T.J., A.W., T.M.Y.); Pediatric Heart Centre, Justus-Liebig-University, Giessen (D.S.); and Division of Thoracic and Cardiovascular Surgery, Hannover Medical School, Hannover (T.B.), Germany

Correspondence to T. Mesud Yelbuz, MD, PhD, Division Pediatric Cardiology, McMaster Children’s Hospital, McMaster University, 1200 Main St W, HSC-3A, Hamilton, ON L8S 3Z5, Canada. E-mail yelbuz{at}mcmaster.ca

Tetralogy of Fallot is still the most common cyanotic heart defect and occurs in 1 in 3600 newborns or in 3.5% of the newborns with congenital heart disease.^1,2 Since the first efforts of surgical repair in the 1950s, operation techniques with right ventricular outflow tract augmentation (RVOTA) and closure of ventricular septal defects have improved continuously. Today, in countries providing a high standard of medical care, patients with uncorrected tetralogy of Fallot presenting classic clinical signs of cyanosis are rare.

A 6-year-old girl from Afghanistan with tetralogy of Fallot, a large malalignment ventricular septal defect, and high-grade infundibular pulmonary stenosis together with a hypoplastic pulmonary system had undergone a left-sided modified Blalock-Taussig shunt in her home country (exact date of surgery could not be determined). No further interventions were performed until the girl was transferred to Germany at 6 years of age. The clinical signs of chronic cyanosis at this time consisted of an extreme clubbing of the digits, especially of the toes, bluish skin, and a deep central cyanosis as indicated by blue lips and a cyanosed tongue (Figure 1A, 1D, and 1G). The oxygen saturation, measured transcutanously, was 65%. The hemoglobin level was 20 g/dL.

View larger version (108K): [in this window] [in a new window]   Figure 1. Images of the face (A through C), fingers (D through F) and toes (G through I) of our patient on admission (A, D, and G) and at 5 weeks (B, E, and H) and 10 months (C, F, and I) after RVOTA. Note the classic clinical signs of chronic cyanosis on admission, consisting of an extreme clubbing of the digits, especially of the toes, bluish skin color, and a deep central cyanosis as reflected in blue lips and a cyanosed tongue (A, D, and G). Clinical improvement was already observed 5 weeks after palliation (B, E, and H) with pinker skin, lips, and tongue at rest and slight regression in clubbing of her digits. However, 11 months after surgery the extreme clubbing of her fingers and toes had resolved completely, as demonstrated clearly in C, F, and I.

Cardiac catheterization and angiography revealed major aortopulmonary collateral arteries (MAPCAs) arising from both the right and left subclavian artery supplying the hypoplastic right and left pulmonary artery, respectively (Figure 2A and 2C and online-only Data Supplement Movies I, II, and III). No residual shunt was visible over the Blalock-Taussig shunt.

View larger version (148K): [in this window] [in a new window]   Figure 2. Still images from angiography (A through D) to clarify cardiac anatomy and morphology of great vessels and MAPCAs in our patient before (A and C) and after (B and D) surgery with RVOTA. A and B show selective angiographies into the proximal left subclavian artery (LSA) in frontal projection. To enhance visualization of MAPCAs arising from LSA supplying the left pulmonary artery, we further processed both images with digital subtraction. A demonstrates the early phase after injection, depicted in white, and the late phase after injection, shown in black. It is the late phase after injection at which MAPCAs and pulmonary arteries are visualized with high contrast. Note the hypoplasia of both the right pulmonary artery (RPA) and left pulmonary artery (LPA). The original angiography (online-only Data Supplement Movie II) shows both early and late phases of opacification successively. B demonstrates the full regression of MAPCAs, initially arising from the left subclavian artery, 11 months after surgery. See also the original angiography without digital substraction, shown in online-only Data Supplement Movie IV, in which vessels arising from the aortic arch are depicted clearly in the early phase of opacification, whereas in the late phase no contrast in MAPCAs is present compared with presurgical angiography, shown in online-only Data Supplement Movie II. C and D show angiography (frontal projection) of the right subclavian artery (RSA) before (C) and after (D) surgery. Note that MAPCAs arising from RSA, localized medial to the right internal mammarian artery (IMA) (C and online-only Data Supplement Movie III), have also fully regressed after RVOTA, whereas IMA can clearly be visualized, as shown in D and online-only Data Supplement Movie V. AscAo indicates ascending aorta; BCT, brachiocephalic truncus.

As the risk of a 1-step repair operation was considered high, antegrade perfusion of the pulmonary system was increased by RVOTA with a Dacron patch adapted from a hemashield prosthesis (Boston Scientific, Natick, Mass), leaving a residual outflow tract obstruction. With this palliative procedure we could achieve an adaptation and consecutive growth of the pulmonary artery system documented by the improvement of the measured indices of McGoon³ and Nakata.⁴ Presurgical McGoon index was 0.84 (normal value >2), and the presurgical Nakata index was 68 mm²/m² (normal value±SD: 300± 30 mm²/m²); at 10 months after surgery, the indices were 1.9 and 176 mm²/m², respectively.

However, 5 weeks after palliative surgery, the girl already showed a rapid clinical improvement with respect to clinical signs of cyanosis (Figure 1B, 1E, and 1H). After 10 months, the extreme clubbing of her fingers and toes showed a complete regression (Figure 1C, 1F, and 1I). Her skin color improved even more, and oxygen saturation increased to 94 to 97%. The hemoglobin level decreased to 13.3 g/dL. Furthermore, at this time, parallel to the impressive clinical improvement, cardiac recatheterization with a selective angiography of the subclavian arteries demonstrated absence of relevant MAPCAs (Figure 2B and 2D; online-only Data Supplement Movies IV and V). Complete regression of all classic signs of cyanosis was achieved with RVOTA only.

Twelve months after palliation with RVOTA, surgical correction was completed by ventricular septal defect closure and resection of the residual subpulmonary stenosis. Postoperative course was uneventful except for a postoperative atrioventricular nodal reentrant tachycardia, treated and controlled successfully by amiodaron. In summary, the clinical course of this patient illustrates that a stepwise approach in a patient with tetralogy of Fallot and hypoplastic pulmonary arteries may enable a rapid clinical recovery and subsequently allow complete repair with considerably lowered perioperative risk.

	Disclosures

Top Disclosures References

 
None.

	Footnotes

 
Dr Bertram and Dr Jack contributed equally to this article.

The online-only Data Supplement can be found with this article at http://circ.ahajournals.org/cgi/content/full/118/16/e665/DC1.

	References

Top Disclosures References

 
1. Hoffman JI, Kaplan S. The incidence of congenital heart disease. J Am Coll Cardiol. 2002; 39: 1890–1900.[Abstract/Free Full Text]

2. Wilson PD, Corres-Villaenor A, Loffredo CA, Ferenc C. Temporal trends in prevalence of cardiovascular malformation in Maryland and the district of Columbia, 1981–1988. The Baltimore-Washington Infant Study Group. Epidemiology. 1993; 4: 259–265.[Medline] [Order article via Infotrieve]

3. Piehler JM, Danielson GK, McGoon DC, Wallace RB, Fulton RE, Mair DD. Management of pulmonary atresia with ventricular septal defect and hypoplastic pulmonary arteries by right ventricular outflow construction. J Thorac Cardiovasc Surg. 1980; 80: 552–567.[Medline] [Order article via Infotrieve]

4. Nakata S, Imai Y, Takanashi Y, Kurosawa H, Tezuka K, Nakazawa M, Ando M, Takao A. A new method for the quantitative standardization of cross-sectional areas of the pulmonary arteries in congenital heart diseases with decreased pulmonary blood flow. J Thorac Cardiovasc Surg. 1984; 88: 610–619.[Abstract]

This Article Extract Full Text (PDF) Data Supplement Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Request Permissions Citing Articles Citing Articles via Google Scholar Google Scholar Articles by Bertram, H. Articles by Yelbuz, T. M. Search for Related Content PubMed PubMed Citation Articles by Bertram, H. Articles by Yelbuz, T. M. Related Collections Pulmonary circulation and disease Other diagnostic testing Pediatric and congenital heart disease, including cardiovascular surgery