**1. Introduction**

252 Congenital Heart Disease – Selected Aspects

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cardiomyopathy with congestive heart failure in children: a multi-institutional

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

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


Thus, patients with these syndromes will have a parallel circulation in which the systemic and pulmonary circulations will be supplied by "mixed" blood.

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


Fontan Surgery: Experience of One Cardiovacular Center 255

Fig. 2. Second Stage: BT shunt has been replaced with bidirectional Glenn procedure.

looking for:

conduit.

*The third stage repair* (Fontan operation), which is performed in children 12 kg or 4 years old,

anastomosing the inferior cava vein to the pulmonary artery and thus reduces the volume load of the single ventricle and complete the creation of a series circulation (4,5,6). Fig. 3.

Fig. 3. Third Stage: (a) Fenestrated Fontan with lateral tunnel or (b) Fontan extra-cardiac

In these patients one treatment option is repair in three stages, as follows:

*The first stage repair* (Norwood operation: Classic or Sano) seeks to:


Fig. 1. First Stage: The Norwood procedure with a Modified Blalock-Taussig shunt or a right ventricle – pulmonary artery shunt (Sano)

*The second stage repair* (Glenn operation), which Takes place 6 to 8 months after-the Norwood is looking for:

anastomosing the superior cava to the pulmonary artery, thus direct systemic venous return to the pulmonary artery, begin to create a circulation in series. Fig 2.

Provide systemic circulation: the right ventricle is used to support the systemic

 Ensure non-restrictive egress of the pulmonary venous return to "bypass" the left ventricle, the atrial septal defect is enlarged or created in the absence of (in case it did

Provide a controlled pulmonary blood supply, creating a shunt between the systemic

Fig. 1. First Stage: The Norwood procedure with a Modified Blalock-Taussig shunt or a right

*The second stage repair* (Glenn operation), which Takes place 6 to 8 months after-the Norwood

anastomosing the superior cava to the pulmonary artery, thus direct systemic venous return

to the pulmonary artery, begin to create a circulation in series. Fig 2.

In these patients one treatment option is repair in three stages, as follows: *The first stage repair* (Norwood operation: Classic or Sano) seeks to:

Create an outflow tract obstruction-free system: the aorta is reconstructed

circulation

not exist)

and pulmonary circulations

ventricle – pulmonary artery shunt (Sano)

is looking for:

Fig. 2. Second Stage: BT shunt has been replaced with bidirectional Glenn procedure.

*The third stage repair* (Fontan operation), which is performed in children 12 kg or 4 years old, looking for:

anastomosing the inferior cava vein to the pulmonary artery and thus reduces the volume load of the single ventricle and complete the creation of a series circulation (4,5,6). Fig. 3.

Fig. 3. Third Stage: (a) Fenestrated Fontan with lateral tunnel or (b) Fontan extra-cardiac conduit.

Fontan Surgery: Experience of One Cardiovacular Center 257

Frecuency Percentage Cumulative Percentage

The number of patients who failed was a total of 8; the cause in 4 of them (50% of total deaths) was low output syndrome which was present in the first 72 hours postoperatively, and the remaining 4 patients died after 72 hours after Fontan. These patients had a progressive deterioration associated with symptoms and signs of heart failure, proteinlosing enteropathy, persistent chylothorax, affecting directly their functional status and no

The end result is shown in Table No. 4, where 47 of them (84%) were discharged and continued in a functional stage I 9n 82.1% of them (Table No. 5). One patient (1.8%) sample was transferred from the Cardiovascular Clinic to another facility outside the country.

Frecuency Percentage Cumulative Percentage

Frecuency Percentage Cumulative Percentage 48 85.7 85.7

Fenestrated Atriopulmonary 2 3.6 3.6 Atriopulmonary not fenestrated 1 1.8 5.4 Fenestrated Extracardiac 30 53.6 58.9 Not Fenestrated extracardiac 16 28.6 87.5 Fenestrated intracardiac 4 7.1 94.6 Not fenestrated intracardiac 3 5.4 100.0

> No 48 85.7 85,7 Yes 8 14.3 100,0

Total 56 100,0

Total 56 100.0

response to medical management. These data are shown in Table No. 3.

Total 56 100.0

Total 56 100.0

Low Output 4 7.1 92.9 Fontan failed 4 7.1 100.0

Discharge 47 84 84 Death 8 14.2 14.2 Transfer 1 1.8 100.0

Table 1. Fontan type

Table 2. Death

Table 3. Cause of death

Table 4. Cause of discharge

Frecuency Porcentage Cumulative Percentage
