Pulmonary Valve Stenosis

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Research Letter

Double Outlet Right Ventricle with Unrelated Ventricular Septal Defect and Pulmonary Stenosis, in Natural Evolution, in a 36-Year-Old Woman

Introduction

The congenital defect, characterized by the exit of both large arteries from the right ventricle (RV), completely or even when overlapping more than 50% of one of the arteries over the interventricular septum, presents variable relationships between these arteries and the ventricular septal defect (VSD), as well as association with other anatomical and functional variables.

In the most common associated defect, the isolated VSD, in a subaortic position or in that unrelated to the large arteries, the functional condition is expressed with a predominance of volume overload of the heart as a whole, increased by pressure overload, causing early heart failure.

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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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Clinicoradiological Correlation

Case 3/2019 – Type IIB Tricuspid Atresia, in Natural Evolution, at 21 Years of Age

Edmar Atik et al. Alessandra Costa Barreto Maria Angélica Binotto

Clinical data

Patient remained asymptomatic from birth until 16 years of age, when he started to show progressive fatigue at exertion, with the use of anti-congestive medication such as furosemide, enalapril, spironolactone, and carvedilol, in addition to warfarin. The diagnosis of heart disease characterized by heart murmur was attained in the first month of life.

Physical examination: good overall status, eupneic, acyanotic, normal pulses in the 4 limbs. Weight: 63 kg, Height: 171 cm, right upper limb blood pressure: 130/90 mmHg, HR: 63 bpm, Sat O₂: 89%.

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Clinicoradiological Correlation

Case 1 / 2019 – Natural Evolution of Double Outlet Right Ventricle with Noncommitted Ventricular Septal Defect and Pulmonary Stenosis in a 28-Year-Old Asymptomatic Woman

Edmar Atik et al. Alessandra Costa Barreto Maria Angélica Binotto

Clinical data

The patient evolved without symptoms from birth, when the diagnosis of heart disease was made, as evidenced by heart murmur. Two years ago, supraventricular extrasystoles caused the use of atenolol, propafenone, and magnesium, without improvement. Infective endocarditis was effectively treated 10 years ago. The patient uses levothyroxine 50 mcg for hypothyroidism.

Physical examination: good overall status, eupneic, acyanotic, normal pulses in the four limbs. Weight: 60 Kg, Height 160 cm, right upper extremity blood pressure: 120 x 70 mmHg, HR: 60 bpm.

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Clinicoradiological Correlation

Case 5/2018 – Severe Pulmonary Valve Stenosis (PVS), Relieved by a Double-balloon Catheter, in a 68-year-old Woman

Edmar Atik et al. Alessandra Costa Barreto Maria Angélica Binotto Luiz Junya Kajita

Clinical data

During a recent clinical evaluation for cholecystectomy surgery, a heart murmur was auscultated and complaint of fatigue at great efforts lasting for some months was reported by the patient. The diagnosis of severe PVS was attained, with a maximum gradient of 160 mmHg obtained at the echocardiography. The patient denied other symptoms and was unaware of the existence of this cardiopathy. She had no history of other morbidities and took vitamin D.

Physical examination: The patient was in good overall condition, eupneic, acyanotic, normal pulses in the 4 limbs. Weight: 70 kg, height: 160 cm, right upper limb blood pressure: 140/80 mmHg, heart rate: 80 bpm, oxygen saturation, 89%. Aorta not palpable in the suprasternal notch.

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Clinicoradiological Session

Case 6 / 2015 – A 27-Year-Old Male Patient with Double Aortic and Pulmonary Valve Lesion after Double Valvotomy in Childhood

Edmar Atik

Laboratory tests:

Electrocardiogram showed normal sinus rhythm and signs of left anterior hemiblock, with no chamber overload, and normal ventricular repolarization. PA: +20º, QRSA: -60º, TA: +40º. QRS complex duration was 0.11″, PR = 0.16″ and QTc = 0.45″ ().

Chest radiograph showed moderately enlarged cardiac silhouette due to enlarged atrial and ventricular arches; the pulmonary vascular network was normal. Cardiomegaly progressed since the surgical correction, and the current cardiothoracic ratio was 0.58 ().

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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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Artigo Original

Prevalence and surgical approach of supravalvular pulmonary stenosis after jatene operation for transposition of great arteries

Marcelo Biscegli Jatene et al. Ieda Biscegli Jatene Patrícia Marques de Oliveira Rafael Aon Moysés … Luis Carlos Bento de Souza Valmir Fontes Nana Miura Antonio Augusto Lopes Miguel Barbero Marcial Adib Domingos Jatene

BACKGROUND: The Transposition of the Great Arteries is the most frequent congenital cyanogenic cardiopathy in the neonatal period, corresponding to 7% of all congenital cardiopathies. Among the operations for surgical treatment, the Jatene operation, with arterial correction, is the treatment of choice. During the late postoperative evolution, some problems were observed, with the most common being the occurrence of supravalvular stenosis at the neopulmonary, regardless of the type of surgical technique used. OBJECTIVE: To study and analyze the prevalence of stenosis, as well as describe the surgical treatment and propose technical maneuvers to prevent its onset. METHODS: Of the 553 patients that underwent surgery, 409 were discharged from the hospital and 281 had late follow-up; 59 (20.9%) presented different degrees of supravalvular pulmonary stenosis and 21 had a mean gradient > 60 mmHg, needing surgical treatment. Depending on the location and anatomy of the stenosis, the surgical treatment consisted of the use of different techniques, such as the enlargement of stenosis areas with bovine pericardium patches, resection of stenotic areas and termino-terminal anastomosis, replacement of retracted patches and synthetic tubes. RESULTS: Twenty patients presented good evolution and only one patient died. CONCLUSION: It can be concluded that the supravalvular pulmonary stenosis, post-Jatene operation for Transposition of Great Arteries, had a prevalence of 20.9%; once identified and with indication for treatment, it can be treated surgically with low mortality levels, through different surgical techniques; to prevent the occurrence of stenosis, ample dissection and release of the pulmonary branches, double anastomoses, large patches of autologous pericardium and careful reconstruction of the aorta are proposed, which prevents the compression of the neopulmonary.

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Artigo Original

Mild pulmonary valve stenosis: the possible spontaneous cure in the natural history of the defect

Edmar Atik

OBJECTIVE: To determine the long-term natural history of patients with mild pulmonary valve stenosis METHODS: Throughout a 24-year evolutive period (1980 a 2004), 83 patients with pulmonary valve stenosis were evaluated, with 29 of them presenting a mild form of the defect. Of these 29, 13 patients had a long-term follow up. The mean age at the first and last follow-up visits was 34 months (1 month to 15 years) and 10.5 years (3 to 24 years), respectively. In addition to evolutive elements, those regarding clinical, electrocardiographic and echocardiographic aspects were also assessed. RESULTS: All patients remained assymptomatic throughout the study. Regarding the pulmonary valve pressure gradient, 5 (38.4%) presented a decrease, 3 (23%) remained unchanged, 4 (30.7%) presented elimination and 1 (7.6%) presented accentuation to a moderate degree of involvement. The mean initial and final gradient was 24.2 mmHg (15-30) and 13.6 mmHg (0-54), respectively. The initial gradients of the 4 patients who achieved spontaneous cure were 30, 19, 30 and 20 mmHg. The systolic murmur and right ventricular overload had a direct correlation to the gradient pressure degree, subdivided at each 10 mmHg. CONCLUSION: The spontaneous cure of the mild pulmonary valve stenosis is possible to achieve, similarly to other acyanogenic anomalies.

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Artigo Original

Short- and long- term results of percutaneous balloon valvuloplasty in pulmonary valve stenosis

Domingos M. Hatem et al. Iran Castro José Carlos Haertel Raul I. Rossi … Paulo Zielinsky Flávio C. Leboute Nara Pomar Maristela Winckler Rogério N. Kersten Carlos Roberto Cardoso Carlos A.M. Gottschall

OBJECTIVE: To assess the long-term results of percutaneous balloon valvuloplasty at a single institution. METHODS: This study comprised 189 patients with pulmonary valve stenosis undergoing percutaneous balloon valvuloplasty from 1984 to 1996, whose mean age was 7.97±9.25 years. The procedure was classified as successful when the RV-PA gradient was reduced to levels 36 mmHg after an effective procedure. RESULTS: After the procedure, the peak-to-peak transvalvular gradient decreased from 70.12±30.06 to 25.11 ±20.23 mmHg (P<0.001). Immediate success was obtained in 148 (78.72%) patients. A later reduction in the gradient to values < 36 mmHg was obtained in 24 other patients previously categorized as unsuccessful. Therefore, percutaneous balloon valvuloplasty was considered effective in 172 (91.01%) patients. Effectiveness increased to 93.53% (159/170) in the cases of typical morphology. Follow-up ranged from 4.39±3 years to 13.01 years. Restenosis was observed in 24 (13.95%) patients. Pulmonary regurgitation was detected in 95.1% of the patients, being more intense than mild in 29.5% of the patients. The probability of maintaining an appropriate result, at any time point, with no restenosis was 92.29% in 2 years, 87.38% in 5 years, 82.46% in 8 years, and 64.48% in 10 years. CONCLUSION: Percutaneous balloon valvuloplasty was effective and safe for the treatment of pulmonary valve stenosis with excellent short- and long-term results.

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Original Article

Cardiac findings in 31 patients with Noonan's syndrome

Débora Romeo Bertola et al. Ae Kim Chong Sofia M. M. Sugayama Lilian Maria José Albano … Jaqueline Wagenführ Regina Lúcia Moysés Claudette Hajaj Gonzalez

OBJECTIVE: To evaluate cardiac findings in 31 Noonan syndrome patients. METHODS: Thirty-one (18 males and 13 females)patients from 26 families affected with Noonan’s syndrome were evaluated from the cardiac point of view with electrocardiography and echodopplercardiography. RESULTS: Twenty patients had some type of cardiac abnormality. The most frequent was pulmonary valve stenosis followed by hypertrophic myocardiopathy, commonly associated with valve defects. Upper deviation of the QRS axis was observed in 80% of these patients. CONCLUSION: In view of the high frequency and diversity of cardiac abnormalities present in Noonan syndrome, cardiac evaluation with electrocardiography and echocardiography should be performed in all patients diagnostically suspected of having this disease.

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