ABSTRACT
Double Outlet Right Ventricle (DORV) is associated to a wide set of cardiovascular anomalies. We report a rare
combination of congenital cardiac malformation of DORV with unrestrictive non-committed interventricular
communication, persistent left superior vena cava, sub pulmonary stenosis and aberrant right Subclavian artery.
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ABSTRACT
We show a brief report of two common arterial trunk cases (CAT) with different arrhythmias and discuss anatomy,
clinical and diagnostic management. The burden of volume and pressure overload of this cardiac malformation
may predispose to different types of arrhythmia before and after surgical repair. Because of labile
hemodynamic state in this group of patients, prompt diagnosis of any arrhythmia is mandatory as the devastating
factor on prognosis. The first patient with a diagnosis of CAT Type II Collett and Edwards (CE) had a particular
history with HIV seropositive mother assuming antiretroviral therapy during pregnancy, who presented hyperbilirubinemia
and liver dysfunction at birth, and re-entry atrial tachycardia after repair. The second patient
had CAT Type I CE with a partial anomalous venous connection of left superior pulmonary vein and uncommon
type of atrial tachycardia with dual AV nodal physiology.
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ABSTRACT
Fetal arrhythmias reach up around 10% of the total third-level perinatal cardiology references. Sustained bradycardia
is defined as a baseline fetal heart rate (FHR) of less than 110 bpm sustained for at least 10 min. The overall
incidence of malignant fetal bradyarrhythmias, such as complete atrioventricular block (AVB) and channellopathies,
is relatively rare, 1:5000 pregnancies, but represents a serious emergency for the gynecologist, neonatologists,
and pediatric cardiologists. Fetal complete AVB is strongly associated with maternal connective tissue
disease, but it can be also associated with congenital heart disease and usually with a poorer prognosis with high
risk of fetal hydrops and abortion. Currently, the treatment of severe fetal bradyarrhythmias is principally pharmacological
and aims to increase the FHR, besides an early resolution of underlying causes, when possible, and a
promptly management of fetal heart failure. Intrauterine electrostimulation nowadays is an experimental pioneering
method, reserved for limited selected cases.
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Three-dimensional (3D) printing anatomical models of complex heart diseases are precious for preoperative planning, anatomical relationships and intraoperative guidance. Pulmonary atresia with ventricular septal defect (PA-VSD) and major aortopulmonary collateral arteries (MAPCAs) is one of the most challenging surgical conditions to manage because of the timing and the technical complexity of the surgery. This complex congenital heart disease has still a poor prognosis, generally less than 50% survival at 2 years. We describe the impact of computerized tomography scan and 3D printing model for decision making in surgical procedure strategy in a newborn with PA-VSD and MAPCAs, underwent to palliative surgery.
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We present a case of a one-day-old newborn, without prenatal diagnosis, referred to our cardiologic intensive care unit in critical condition presenting sub-cyanosis and peripheral oxygen saturation of 80%. Echocardiography diagnosis was tricuspid valve dysplasia with severe regurgitation, functional pulmonary valve atresia with intact ventricular septum and reversal flow in the large patent ductus arteriosus (PDA). Chest X-ray showed severe cardiomegaly and wall to wall heart. Prostaglandin E1 infusion was started once after birth. After few days, clinical conditions progressively worsened because of right heart failure; a first pharmacological approach to close PDA failed and surgery ligation of PDA was necessary to restore anterograde pulmonary flow and heart size.
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A newborn without prenatal diagnosis, with bronchial and abdominal situs inversus in levocardia, was referred to our hospital for accurate evaluation; echocardiography showed venoatrial connections in mirror-image arrangement, atrioventricular (AV) discordance, and double-outlet right ventricle (DORV). Additional cardiac malformations were double upper caval district, atrial communication, subpulmonary interventricular communication, and moderate subvalvular and valvular pulmonary stenosis. Few days after birth, the patient presented low oxygen saturation and the heart team decided for a palliative surgery. We describe a very rare case in a newborn with bronchial-abdominal mirror imagery, AV discordance, and DORV in levocardia.
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Farruggio S1, Capodanno D2, Calvi V2, Silvia A3, Campisi M3, Di Mambro C1, Agati S1, Gitto P1, Caruso E1*
1 Mediterranean Pediatric Heart Center “Bambino Gesù”, San Vincenzo Hospital, Contrada Sirina 98039, Taormina (ME), Italy.
2 Division of Cardiology, CAST, P.O. “G. Rodolico”, Azienda Ospedaliero-Universitaria “Policlinico-Vittorio Emanuele,” University of Catania, Catania, Italy.
3 Pediatric Cardiology Unit, Maternal-Neonatal Department, Azienda di Rilievo Nazionale e Alta Specializzazione “Garibaldi”, Catania, Italy.
*Corresponding Author: Dr. Elio Caruso, Mediterranean Pediatric Heart Center “Bambino Gesù”, San Vincenzo Hospital, Contrada Sirina 98039 Taormina (ME), Italy,
Prenatal diagnosis of congenital heart disease (CHD) allowed reduction significantly perinatal morbidity and mortality. The development of new ultrasound technologies and above all the greater experience of the operator permitted us to achieve excellent levels of accuracy in prenatal echocardiography diagnosis and to plan the best therapeutic strategy as well as the delivery in a third-level hospital equipped with cardiac surgery and neonatal intensive care.
The great interest surrounding the prenatal diagnosis of cardiac defects is justified by the epidemiological relevance of the problem, since the incidence is 1% on all live births [1], a potentially even greater percentage if we consider spontaneous abortions and stillbirths.
In Europe, a prevalence of CHD is estimated at 7.2 children per 1,000 live births and 8 per 1,000 considering TOP and fetal deaths [2]. Among premature births, excluding the patency of the ductus arteriosous and atrial septal defects, it reaches 12.5 per 1000 live births [3]. CHD is the major cause of infant mortality, about 3% of all infant deaths and 46% of deaths due to congenital malformations.
CHD represent the main cause of infant death in the western world [4].
Prenatal diagnosis has a strong impact on therapeutic terminations of pregnancy (TOP) and on reducing the prevalence of CHD in live births. A recent analysis [5] evaluated international trends (Europe, America and Asia) on prenatal diagnosis of complex cardiac anomalies and found considerable variability in the prenatal diagnosis rate of heart disease ranging from 13% to 87%. Therefore, the purpose of this study is to analyze the impact of the prenatal echocardiographic diagnosis on our regional population and the outcome of the pregnancy and treatment at birth.
About 18-25% of affected children in natural history die within the first year of life, while 4% of survived do not exceed 16 years [6]. Overall, the CHD is distributed homogeneously in the two genders (males 48.7%, females 51.3%) [7], while there is a different incidence of some specific heart diseases between various ethnic groups. Furthermore, it has been shown that CHD is more frequent in fetuses of twin pregnancies than in single pregnancies [8].
According to a recent meta-analysis [9] in the context of CHD, someone is decidedly more frequent than others, such as ventricular septal defect (VSD), atrial septal defect (ASD), patent ductus arteriosous (PDA), pulmonary stenosis (PS), tetralogy of Fallot (TOF) and transposition of great arteries (TGA). The trend of CHD, without considering septal defects, shows a progressive increase over time with an almost doubled prevalence of obstructions in the right ventricular outflow tract (RVOT) and a reduction of about one third in obstructions in the left ventricular outflow tract (LVOT), probably related to increased TOP due to the diagnosis of CHD such as hypoplastic left ventricular syndrome (HLHS). However, these data are influenced by multiple variables: method of assessment, method of diagnosis, population studied, subjects examined, verification of the diagnosis even after birth, assessment period, inclusion and exclusion criteria, type of classification [10].
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Farruggio S1, Khorgami MR2, Zanai R1, Calvaruso D1, Ortiz Ruiz DA1, Agati S1, Di Mambro C1, Caruso E1*
1. Department of Pediatric Cardiology, Mediterranean Pediatric Cardiology Center “Bambin Gesù”, San Vincenzo Hospital, Taormina (ME), Italy.
2. Cardiac Electrophysiology Research Center, Rajaie Cardiovascular Medical and Research Center, Iran University of Medical Sciences, Tehran, Iran
*Corresponding Author: Dr. Elio Caruso, Department of Pediatric Cardiology, Mediterranean Pediatric Cardiology Center “Bambin Gesù”, San Vincenzo Hospital Contrada Sirina 98039 Taormina (ME), Italy.
We report a very rare case of ventricular tachycardia (VT) in a two-month-old female infant, with congenital heart disease (CHD), vascular and anorectal malformations.
She was born full-term by cesarean section at 38 weeks of gestational age, from unrelated parents with a family history negative for CHD. At birth she presented good clinical condition, APGAR of 9 at 1’ and 5’ minutes after delivery, normal blood pressure, her body weight at birth was 3.040 kg with 93% of O2 blood saturation (O2 BS), and physical examination showed a grade 3/6 holosystolic murmur and anorectal atresia.
Echocardiogram showed atrial septal defect ostium secundum type shunting left-to-right (Fig. 1 A), patent ductus arteriosus (PDA) shunting aorta-to-pulmonary artery, left-superior caval vein (LSCV) draining directly in the left atrium (Fig. 1 D) with unroofed coronary sinus, and subaortic perimembranous ventricular septal defect (VSD) wide about 5 mm (Fig. 1 B-C) shunting mainly left-to-right (Fig. 1 C).
The patient after one day from the birth underwent to colostomy surgery in another hospital and about after two months was referred to our intensive care unit (ICU) for dyspnea and frequent premature ventricular contractions (PVCs) during hospitalization in the other center. Baseline ECG made in the other hospital showed a sinus rhythm (SR) with a heart rate (HR) of 150 beats per minute (bpm), high P-wave voltage (> 0.25 mV in the limb leads), normal PR interval (120 ms) and cardiac axis, increased QRS voltage in precordial leads, nonspecific ST-T wave changes. ECG monitor in ICU showed a suspected supraventricular tachycardia (SVT) with narrow QRS, then a first therapeutic approach was done with iv adenosine bolus at the dosage of 0,1 mg/kg increasing dosage to 0,2 mg/kg until 0,4 mg/kg without resolution of arrhythmia. The patient was pale, dyspnoic with good blood pressure. A transesophageal electrophysiological study was performed, after informed consent obtained from parents, on the doubt of an SVT refractory to adenosine. Atrial stimulation started by 10 mA for 10 ms with an HR faster than the baseline and adjusted according to the stimulus threshold (Esodik 6 fr quadripolar catheters (FIAB programmable cardiac stimulator 8817) was attempted in ICU, without sedation, showing atrial capture with high rate overdrive without interrupting the arrhythmia. Esophageal atrial pacing was done with a shorter cycle length than RR interval, as a result, we observed the QRS duration became narrower with overdrive pacing, later, after stopped atrial pacing, we expected the QRS duration became wider due to the behavior of the cardiac conduction system with atrial pacing. Therefore, after the last paced beat, the ventricular arrhythmia reappears with the same cycle length (Fig. 2 B). After these quick attempts in our ICU, 12 lead ECG showed tachyarrhythmia with a QRS complex wide 0,08 ms (high age limit) with atrial retroconduction and HR of 280 bpm (Fig. 2 A). A few minutes later ECG showed wide QRS tachycardia alternated to SR and ventricular bigeminy (Fig. 2 C). A differential finding between SVT and VT is the similarity of bigeminy PVCs during sinus rhythm with QRS morphology during tachycardia (Fig. 2 A-C). Therefore, the final diagnosis was VT for the followed findings: VA dissociation (Fig. 3) with ventricular rates exceeding atrial rates (AV ratio < 1:1), QRS duration at the high upper limit for age (0,08 sec) with inferior axis configuration, and morphology of QRS complex suggested a monomorphic VT originating from right ventricular outflow tract (RVOT-VT) [1].
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