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
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.
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.
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.
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.
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.