**14. References**


(increased Po2, increased systemic blood pressure, and improved pH) are achieved, the dose should be reduced step-by-step to 0.01µg/kg/min. When there is no effect with the initial

If echocardiography is not immediately available, the clinician caring for a newborn with possible cyanotic heart disease should not hesitate to start a prostaglandin infusion ( for a possible ductal-dependent lesion). Because of the risk of hypoventilation associated with prostaglandins, a practitioner skilled in neonatal endotracheal intubation must be available. Three common side effects of Prostaglandin E1 IV infusion are apnea (12%), fever (14%),

The evaluation of the cyanotic neonate should be done in an algorithmic manner that focuses on evaluation and management of the most life-threatening disease processes first.The hyperoxia test should be utilized early in the evaluation of these patients to assist in the differentiation and categorization of the cyanotic event. Be careful to obtain a detailed history of the prenatal, birth, and postnatal periods, as physicians will often be able to narrow the differential by the history alone. Neonates may decompensate very quickly, and

[1] Baba K, Ohtsuki S, Kamada M, Kataoka K, Ohno N, Okamoto Y, et al. Preoperative

[2] Fricker FJ. Hypoplastic Left Heart Syndrome – Diagnosis and early management.

[3] Hoffmann GM, Ghanayem NS, Kampine JM, Berger S, Mussatto KA, Litwin SB, et al.

[5] Lewis AB, Freed MD, Heymann MA, Roehl SL, Kensey RC. Side effects of therapy with

[6] Thiebaud B, Michelakis E, Wu XC, Harry G, Hashimoto K, Archer SL. Sildenafil

phosphodisesterase and activating BK Ca Channels. Ped Res 2002;52:19-24. [7] Rychik J, Rome JJ, Collins MH, DeCampli WM, Spray TL. The hypoplastic left heart

[8] Atz AM, Feinstein JA, Jonas RA. Preoperative management of pulmonary venous

histopathology and outcome. J Am Coll Cardiol 1999;34:554-60.

management for tricuspid regurgitation in hypoplastic left heart syndrome. Ped

Venous saturation and the anaerobic threshold in neonates after the Norwood procedure for hypoplastic left heart syndrome. Ann Thorac Surg 2000;70:1515-21. [4] Singh GH, Fong LV, Salmon AP, Keeton BR. Study of low dosage prostaglandin – usages

prostaglandin E1 in infants with critical congenital heart disease. Circulation

Reverses O2 constriction of the rabbit Ductus Arteriosus by inhibiting Type 5

syndrome with intact atrial septum: atrial morphology, pulmonary vascular

hypertension in hypoplastic left heart syndrome with restrictive atrial septal defect.

preparations for a life-saving emergency should be made as soon as possible.

starting dose, it may be increased to 0.4 µg/kg/min.

and flushing (10%).

**13. Summary** 

**14. References** 

Internat 2009;51:399-404.

NeoReviews 2008;9:253.

Am J Cardiol 1999;83:1224-8.

1981;64: 893-8.

and complication. Eur Heart J 1994;15:377-81.


**10** 

*Cardiovascular Clinic* 

*Colombia* 

**Fontan Surgery: Experience** 

**of One Cardiovacular Center**

Monica Guzman, Juan Marcos Guzman and Miguel Ruz

In order to establish a normal, in-series circulation physiologically, very different from the circulation in parallel which the children are born with single ventricle, doctors Fontan and Baudet (2) and Kreutzer (3) concurrently developed surgical treatment of patients with

Management strategies for patients with functional single ventricle required a staged group of procedures where the ultimate goal is to have a single ventricle with a working pressure and volume close to normal as well as normal systemic oxygen saturation (1). It is known that both vascular development and lung maturation are essential for achieving benefits of cavopulmonary connection; the time of surgery has been defined arbitrarily and even more

Single ventricle or univentricular heart anatomically or physiologically characterized by: Both atrioventricular valves attached to a single systemic ventricular chamber

Thus, patients with these syndromes will have a parallel circulation in which the systemic

Pulmonary venous return directed to the right atrium through a patent foramen ovale,

In these patients, ductal closure would result an inadequate systemic perfusion and

The following are the physiological characteristics of hypoplastic left heart syndrome:

atrial septal defect or rarely, total anomalous pulmonary venous drainage

 The right ventricle supplies the systemic and pulmonary circulation in parallel Retrograde blood flow from the ductus arteriosus to the coronary arteries

metabolic acidosis with progressive coronary ischemia, and death.

tricuspid atresia to achieve a passive flow through the pulmonary vascular bed (1).

time to transition between partial to total cavopulmonary connection (1).

The essential characteristics of hypoplastic left heart syndrome syndrome are:

Severe stenosis or atresia of the atrioventricular valves

2. Underdeveloped severely hypoplastic left ventricular

and pulmonary circulations will be supplied by "mixed" blood.

Mixed systemic and pulmonary venous return in right atrium

 There is no separation between the ventricles One of the ventricles is hypoplastic or absent

1. Stenosis or atresia of the mitral valve

5. Ductal dependent for systemic blood flow

3. Stenosis or aortic valve atresia

Non-functional left ventricle

4. Small aortic root

**1. Introduction** 

