Fontan Procedure – ABC CARDIOL

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Research Letter

Left Heart Hypoplasia Evolving Up to 21 Years, After Total Cavopulmonary Operation Performed at 5 Years of Age

Introduction

Hypoplasia of the left heart is characterized by very small dimensions of the left cardiac cavities, represented by the left atrium, mitral valve, left ventricle, aortic ring and ascending aorta. Systemic circulation thus becomes dependent on the ductus arteriosus with reverse flow from the hypertensive pulmonary artery to the descending aorta, and also to the ascending aorta. Coronary circulation is also dependent on this reverse flow, which is why there is a high chance of myocardial infarction, even in the first days of life. It is easy to imagine that, on decreased pulmonary arterial pressure or ductus arteriosus caliber, there is an unfavorable evolution towards low cardiac output. The right heart is enlarged and atrial septal defect invariably accompanies this defect. When this atrial defect appears to be of reduced dimensions, known as restrictive, it causes retrograde pulmonary hypertension. On the other hand, when larger, it may favor the faster involution of pulmonary hypertension, with congestive symptoms and low output. In the rare cases of intact atrial septum, anomalous pulmonary vein drainage remains in order of maintenance and viability of circulation. Left heart hypoplasia is the fourth most common heart disease in the neonatal period, with high mortality, even in this period, and corresponds to 1.5% of all congenital heart diseases.

How it is externalized and evolves: Dynamic instability favors the advent of very early signs and symptoms in life, in the first hours or days of life. These signs are characterized by congestive condition (with large atrial defect) or low systemic output (decreased pulmonary arterial pressure and/or constriction of the ductus arteriosus), with pallor, dyspnea, discomfort, irritability and variable cyanosis. The most favorable picture corresponds to the one with restrictive atrial septal defect, which, in view of the maintenance of pulmonary hypertension, the cardiac output is maintained at a satisfactory level.

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Clinicoradiological Correlation

Case 4/2020 – Prolonged Time (38 Days) of Bilateral Pleural Effusion after Cavopulmonary Surgery, Relieved by Embolization of Systemic-Pulmonary Collateral Vessels, in a 40-Month-Old Child with Complex Heart Disease

Edmar Atik et al. Raul Arrieta Fernando Antibas Atik

Clinical Data

The fetal diagnosis of complex cardiac anomaly (Double-outlet right ventricle, severe pulmonary stenosis due to anterior deviation of the infundibular septum, trabecular interventricular septal defect and hypoplasia of the left ventricle and mitral valve) was confirmed shortly after birth with severe hypoxia, relieved by prostaglandin E1 administration and dilation of the ductus arteriosus by percutaneous stent. With the recurrence of more severe hypoxia, the bidirectional Glenn operation was performed at 9 months of age. Good patient evolution was observed up to 39 months, when total cavopulmonary (Fontan) operation was performed due to the recurrence of hypoxia with 70% oxygen saturation. The patient received propranolol and ASA up to the last intervention.

Physical examination: good general status, eupneic, marked cyanosis, normal pulses in the 4 limbs. Weight: 16.35 Kgs, Height: 91 cm, BP: 90 x 60 mm Hg, HR: 116 bpm, O₂Sat: 70%, Hg = 15.5 g, Hct = 55%.

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Original Article

Outcomes of the Conversion of the Fontan-Kreutzer Operation to a Total Cavopulmonary Connection for the Failing Univentricular Circulation

Gabriel Carmona Fernandes et al. Guilherme Viotto Rodrigues da Silva Luiz Fernando Caneo Carla Tanamati … Aida Luiza Ribeiro Turquetto Marcelo Biscegli Jatene

Abstract

Background:

The Fontan-Kreutzer procedure (FK) was widely performed in the past, but in the long-term generated many complications resulting in univentricular circulation failure. The conversion to total cavopulmonary connection (TCPC) is one of the options for treatment.

Objective:

To evaluate the results of conversion from FK to TCPC.

Methods:

A retrospective review of medical records for patients who underwent the conversion of FK to TCPC in the period of 1985 to 2016. Significance p < 0,05.

Results:

Fontan-type operations were performed in 420 patients during this period: TCPC was performed in 320, lateral tunnel technique in 82, and FK in 18. Ten cases from the FK group were elected to conversion to TCPC. All patients submitted to Fontan Conversion were included in this study. In nine patients the indication was due to uncontrolled arrhythmia and in one, due to protein-losing enteropathy. Death was observed in the first two cases. The average intensive care unit (ICU) length of stay (LOS) was 13 days, and the average hospital LOS was 37 days. A functional class by New York Heart Association (NYHA) improvement was observed in 80% of the patients in NYHA I or II. Fifty-seven percent of conversions due to arrhythmias had improvement of arrhythmias; four cases are cured.

Conclusions:

The conversion is a complex procedure and requires an experienced tertiary hospital to be performed. The conversion has improved the NYHA functional class despite an unsatisfactory resolution of the arrhythmia.

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Clinicoradiological Session

Case 1/2017 – Percutaneous Repair of Right Atrioventricular Valve Insufficiency and Blalock-Taussig Shunt after Fontan Operation in Single Ventricle

Edmar Atik et al. Renata Sá Cassar Raul Arrieta

Clinical data

Twenty-seven-year old male patient reporting tiredness during exercise for three years, after total cavopulmonary connection with extracardiac conduit fenestration and closure with stiches of the free end of right atrioventricular valve (AV) for severe failure in double inlet left ventricle, pulmonary atresia, and aorta arising from rudimentary right ventricle. The patient had undergone right and left Blalock-Taussig shunts at 17 days and 9 months, respectively, and bidirectional Glenn shunt at 19 years old. The patient had arterial oxygen saturation of 84-88% during exercise and 93% at rest.

Physical exam: eupneic, cyanotic, normal pulse rate, with no jugular vein dilation. Weight: 61 Kg, Height: 163 cm, BP: 110/70 mm Hg, HR: 97 bpm, oxygen saturation = 87%. The aorta was palpable in the supra-sternal notch (grade 2).

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Clinicoradiological Session

Case 1/2014 – 24-Year-Old Man with Left Single Ventricle in Chronic Hypoxia

Edmar Atik et al. Marcelo B. Jatene Patricia O. Marques Fabiana Passos Succi

Complementary tests

Electrocardiogram showed sinus rhythm and signs of biatrial and left ventricle overloads. P wave was peaked in I, V2-5, and extended in II, F, V5-6, with negative deflection in V1 and V2. The QRS complex was of RS morphology in V1 and Rs in V6. AQRS: +120º, AT: -60º, AP: +40º.

Chest radiography showed an enlarged heart of mild degree (cardiothoracic ratio: 0.54) with left long ventricular arch and also a long left medium arch. The pulmonary vascular bed was increased ().

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Case Report

Association of plastic bronchitis to protein-losing enteropathy after fontan operation

Vanessa Alves Guimarães et al. Edmar Atik Jussara Bianchi Castelli Nana Miura Ikari … Ana Maria Thomaz Antonio Augusto Barbosa Lopes

We report an unusual case of association of plastic bronchitis (PB) to protein-losing enteropathy (PLE) in a girl of 4 years and 9 months of age with double inlet single left ventricle and ventriculoarterial concordance. submitted to total cavopulmonary surgery. with an intracardiac lateral tunnel at the age of three. The elimination of the 10 cm fibrin bronchial mold (PB) and the alpha-1-antitrypsin elevation of 52 mg/g in feces had both become outstanding. Using sildenafil. the thoracic duct ligature and the cardiac transplant were programmed in case of continuity of the process.

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Left Heart Hypoplasia Evolving Up to 21 Years, After Total Cavopulmonary Operation Performed at 5 Years of Age

Introduction

Case 4/2020 – Prolonged Time (38 Days) of Bilateral Pleural Effusion after Cavopulmonary Surgery, Relieved by Embolization of Systemic-Pulmonary Collateral Vessels, in a 40-Month-Old Child with Complex Heart Disease

Clinical Data

Outcomes of the Conversion of the Fontan-Kreutzer Operation to a Total Cavopulmonary Connection for the Failing Univentricular Circulation

Abstract

Background:

Objective:

Methods:

Results:

Conclusions:

Case 1/2017 – Percutaneous Repair of Right Atrioventricular Valve Insufficiency and Blalock-Taussig Shunt after Fontan Operation in Single Ventricle

Clinical data

Case 1/2014 – 24-Year-Old Man with Left Single Ventricle in Chronic Hypoxia

Complementary tests

Association of plastic bronchitis to protein-losing enteropathy after fontan operation

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