Malalignment Ventricular Septal Defect Research Articles

The Role of Cardiac MRI and Late Gadolinium Enhancement Grading Protocol in Evaluation of the Right Ventricle in Patients with Repaired Fallot Tetralogy

Abstract Background Tetralogy of Fallot represents 7–10% of congenital heart diseases occurring in 0.5/1000 live births and is the second most common form of complex congenital heart diseases. The four elements of Tetralogy of Fallot: (a) sub-pulmonary obstruction resulting in (b) right ventricle hypertrophy, and (c) malalignment ventricular septal defect resulting in (d) apparent overriding of the aorta over both ventricles. Primary cardiac repair for Fallot Tetralogy patients includes right ventricular out flow tract widening interventions and ventricular septal defect closure. For such patients right ventricular assessment in the postoperative period is essential in aiding further management planning. The purpose of this study is to emphasize the role of cardiac MRI in functional and structural assessment of the right ventricle in Fallot tetralogy patients after surgical management. Results The current study included 30 Fallot Tetralogy patients underwent cardiac MRI after surgical management. The majority of patients 26 out of 30 (86.6%) showed right ventricular dilatation. Four patients had right atrial dilatation. Less than one-third of patients 8 out of 30 (26.6%) had right ventricular systolic dysfunction. The majority of patients 29 out of 30 (96.6%) had main pulmonary artery regurgitation. More than half of the patients 18 out of 30 (60%) had main pulmonary artery stenosis. The majority of patients 25 out of 30 (83.3%) had right ventricular outflow tract late gadolinium enhancement and 18 patients (60%) had base of interventricular septal enhancement. Conclusions MRI is an extremely useful imaging method for functional and structural assessment of the right ventricle after surgical repair of Fallot tetralogy patients with absence of ionizing radiation.

Clinical Presentation and Therapy of Tetralogy of Fallot and Double-Outlet Right Ventricle.

Tetralogy of Fallot (TOF) is the most common cyanotic heart defect. TOF consists of the combination of four anomalies (Fig. 35.1): (1) a large malalignment ventricular septal defect, (2) an obstruction of the right ventricular outflow tract (usually infundibular and valvular pulmonary stenosis with a small pulmonary valve annulus and supravalvular stenosis, (3) an aorta that "overrides" the ventricular septal defect, and (4) right ventricular hypertrophy. TOF represents 4-8% of congenital heart defects. Specific variations of TOF include all forms of pulmonary atresia with VSD and absent pulmonary valve syndrome. In addition, the left and right main pulmonary arteries may be stenotic or hypoplastic. In these cases, there may be major aortopulmonary collateral arteries (MAPCAs) which are vessels arising from the aorta or the subclavian arteries that supply segments of the pulmonary arterial tree. Additional variations include an ASD (Pentalogy of Fallot), a right aortic arch, and coronary abnormalities.

Aortic Arch Reconstruction Using Nonvalved Femoral Vein Homograft in High-Risk Neonates.

Aortic arch obstruction is often present with complex concomitant congenital heart defects (CHDs). The use of nonvalved femoral vein homograft (FVH) to reconstruct the aortic arch has distinct surgical advantages, including simplified reconstruction. We present an intraoperative video of a Yasui procedure utilizing FVH for aortic reconstruction in a 12-day-old (2.2 kg) neonate with right ventricular outflow tract obstruction, malalignment ventricular septal defect, aortic valve atresia, aortic arch hypoplasia, atrial septal defect, and ductal dependent systemic circulation. Further, we report outcomes for a series of three additional neonatal patients with complex CHD and aortic arch obstruction who underwent FVH arch reconstruction.

The Outcome of Surgical Pulmonary Artery Banding in Patient with Complex Cyanotic Heart Disease Truncus Arteriosus Type I with Malalignment Ventricular Septal Defect: A Case Report

The Outcome of Surgical Pulmonary Artery Banding in Patient with Complex Cyanotic Heart Disease Truncus Arteriosus Type I with Malalignment Ventricular Septal Defect: A Case Report

Repair of pseudoaneurysm in extra-anatomic aortic arch bypass graft.

Repair of pseudoaneurysm in extra-anatomic aortic arch bypass graft.

A new association of Oculoauriculovertebral spectrum and persistent fifth aortic arch -double lumen aorta: a case report

BackgroundOculo-auriculo-vertebral spectrum is a heterogeneous group of genetic disorder, also known as Goldenhar Syndrome, which has several phenotypic features including craniofacial anomalies, cardiac, vertebral and central nervous system defects. Cardiovascular anomalies include ventricular septal defects, atrial septal defects, patent ductus arteriosus, Tetralogy of Fallot, double outlet right ventricle, aberrant right subclavian artery, coarctation of aorta, transposition of the great arteries, double inlet left ventricle, cor triatriatum, pulmonary artery stenosis, aortic stenosis, persistent left superior vena cava, partially or totally abnormal pulmonary venous return and bicuspid aortic valve. Persistent fifth aortic arch, also named as double lumen aortic arch, is a very rare cardivascular anomaly and usually associate other cardiac defects.Case presentationWe present a 7 month old patient with oculo-auriculo-vertebral spectrum signs as facial asymmetry, short neck, choanal atresia, cleft palate, bilateral preauricular skin tags, bilateral hypoplastic ear lobes, epibulbar dermoid cyst, rib, vertebrae and cardiovascular anomalies. Cardiovascular anomalies detected with echocardiography and computed tomography were malalignment ventricular septal defect and double lumen aorta, known as persistent fifth aortic arch.ConclusionVarious cardiovascular anomalies may accompany Goldenhar Syndrome. We present a case with persistent fifth aortic arch and Oculo-auriculo-vertebral spectrum and this is a new association that was not reported before in the literature.

Brain Abscess Due to Neglected Tetralogy of Fallot: A Case Report

Backgroiund: Tetralogy of Fallot (TOF) is a type of congenital heart disease accounts for about 10% of all congenital cardiac deformities, and is the most common cyanotic lesion after the first year of life. The ideal age for correction of tetralogy of Fallot is still under discussion. Non-cardiac manifestation due to oxygen deficiency has to be aware of in cyanotic patient is brain abscess, because of the right-left shunt in long standing TOF.
 Objectives: To report a case of neglected TOF.
 Case And Discussion: A one-year-old male presented to Murni Teguh Memorial Hospital with the chief complaint of stiffed neck for the past 3 days and got fever for 10 days before admitted, with nausea and vomiting. He was diagnosed with Tetralogy of Fallot through an echocardiography examination when he was 2 months old, but never go through any medical nor surgical treatment. The boy responded to verbal, no eye contact, high grade fever, stiffed neck, Kernig sign and Brudzinski sign was found, and the right extremities were spastic, clubbing finger. On cardiac examination, there was systolic murmur grade ¾ in the left second intercostal space. Echocardiography evaluation revealed dilated RA-RV, no PDA shunt, large mal alignment ventricular septal defect, overriding aorta > 50%, right ventricular hypertrophy, and severe infundibular pulmonary stenosis, consistent with Tetralogy of Fallot. During hospitalization, there were several episodes of seizure and decreased of consciousness, brain CT investigation was done. The abscess was evacuated and a ventriculoperitoneal shunt was performed. One week after the operation, the patient developed abdominal distention, green bile like vomiting, decreased bowel movement and soon muscular defense. From the plain abdominal x-ray and CT revealed peritonitis and intestinal obstruction. An emergency laparotomy was performed, followed by adhesiolysis and jejunostomy due to jejunal perforation. The boy passed away after several episodes of septic shock.
 Conclusions: We would like to emphasis the consequence of the neglected treatment in infant with TOF.

Truncus Arteriosus - modified Van Praagh\u2019s Type 3A and Anesthesia: a case report.

BackgroundOne of the rare complex congenital anomalies is truncus arteriosus—modified Van Praagh’s type 3A. Survival of this type of truncus arteriosus child beyond infancy without surgical treatment is unreported. Anesthesiologists do anesthetize children with complex congenital heart disease during the cardiac catheterization study. The final diagnosis of such children is often made after the anesthesia and cardiac catheterization study. We report a 12-year-old with truncus arteriosus with absent right pulmonary artery and main pulmonary artery with multiple Major Aorto-Pulmonary Collateral Arteries. (MAPCAs) for the right lung, who is surviving without surgical treatment.Case presentationA 12-year-old girl was brought by her parents to Meenakshi Hospital at Thanjavur (India) with complaints of shortness of breath during respiratory infection. The patient was diagnosed to have congenital heart disease at 6 years of age and not on any treatment. There was no history of cyanotic spell. Her echocardiography revealed tetralogy of Fallot, situs solitus, levocardia, large mal-aligned ventricular septal defect with bidirectional shunt, VSD size 12 mm, pulmonary atresia, moderate tricuspid regurgitation (TR pressure gradient, 103 mmHg), thickened aortic valve, grade II aortic regurgitation, right ventricular hypertrophy, intact interatrial septum, dilated right atrium/right ventricle, dilated coronary sinus, and persistent left superior vena cava, good biventricular function 65%, multiple MAPCAs, no coarctation of aorta, normal veno atrial, atrio-ventricular connections, normal pulmonary venous drainage, and no pericardial effusion. She underwent cardiac catheterization study for further evaluation under anesthesia. Her final diagnosis was truncus arteriosus with absent right pulmonary artery and main pulmonary artery with multiple MAPCAs for right lung, (truncus arteriosus—modified Van Praagh’s type 3A).ConclusionAn anesthesiologist may be encountering such patients during cardiac catheterization study or emergency non-cardiac surgery, where an understanding of the complex anatomy (the aorta, left pulmonary artery, coronary artery, all arising from the common arterial trunk, the truncus arteriosus) and the physiology of their circulation would help in safe anesthesia. From our report, we conclude intra venous ketamine along with regional analgesia would be safe for sedating such patients coming for cardiac catheterization study.

Modified Yasui Operation Using Cryopreserved Femoral Vein Homograft

The Yasui operation is indicated in an interrupted aortic arch and a posterior malaligned ventricular septal defect with a narrow subaortic region. We present a modification of the Yasui procedure in which the aortic reconstruction was simplified using a nonvalved cryopreserved femoral vein homograft to connect the pulmonary artery to the descending aorta. A side-to-side anastomosis was performed between the femoral vein homograft and ascending aorta to complete the neoaortic reconstruction. After baffling the left ventricle to the pulmonary artery with a patch, a valved segment from the same femoral vein homograft was used to restore continuity of the right ventricular outflow.

P20.09: Tetralogy of Fallot and malalignment ventricular septal defect detected at early echocardiography

P20.09: Tetralogy of Fallot and malalignment ventricular septal defect detected at early echocardiography

Ischemic Stroke In infant With Tetralogy of Fallot

Objectives: Although stroke is often viewed as occurring primarily in the elderly, it also strikes infants, children, young adults. Stroke in children are rare but increasingly important conditions due to the severity of their complications and diverse diagnostic differentials. It is not yet clearly understood and with multifactorial etiologies. When it comes to stroke, children are not just little adults. Stroke risk factors, symptoms, prevention efforts, and treatment are often different in children than in adults. Up to 80 % of children with ischemic stroke have cardiovascular disease. Case report: RS, An infant, aged two and half years old was admitted to DR. M. Djamil hospital Padang, with right hemiplegy and developed aphasia after activity. One year ago, he had history of cyanotic skin but neglected by his parents and never sought medical treatment. Physical findings on this patient are cyanosis and clubbing fingers. Cardiac auscultation revealed a pansystolic murmur grade III-IV best heard at the upper left sternal border. Chest radiograph showed a characteristic “bootshaped” heart, which is a reflection of Right Ventricular (RV) hypertrophy. The ECG showed right axis deviation. Brain CT Scan revealed a large hypodense lesion on the left cerebral hemisphere suggested as large brain infarction. Echocardiogram revealed tetralogy of fallot with a larged malaligned ventricular septal defect (VSD) with 60 % aortic override. Patient was managed conservatively. After two weeks of admission, patient was discharged with improvement.Conclusion : Ischemic stroke in this infant with tetralogy of fallot occurred via paradoxical embolism mechanism.

How Small Is Too Small? Decision-Making and Management of the Small Aortic Root in the Setting of Interrupted Aortic Arch.

Interrupted aortic arch is commonly associated with a posterior malalignment ventricular septal defect (VSD) and left ventricular outflow tract (LVOT) hypoplasia. Standard repair is carried out in the neonatal period and includes re-establishing arc continuity and VSD closure. Reintervention on the LVOT for obstruction is a common and an ongoing source of morbidity and mortality. A variety of preoperative echocardiographic measurements have been identified to identify patients at risk for developing LVOT obstruction but an aortic valve annulus dimension (mm) < patient's weight (kg) + 1 mm is a reasonable threshold to identify a patient at increased risk for future LVOT reintervention. Prophylactic direct approaches to prevent future LVOT obstruction include myectomy/myotomy and left-sided placement of the VSD patch but do not reliably prevent late LVOT obstruction. Patients amendable to a biventricular repair but with important LVOT hypoplasia are probably best served with a Yasui operation, either as a primary operation or staged with a Norwood procedure. In the case of complex redo operations, a Ross-Konno provides another valuable option for a durable repair. Though smaller preoperative LVOT structures predict the need for reoperation, careful preoperative planning may minimize the need for LVOT reintervention and improve long-term survival.

Truncal Half Turn and Senning Operation: Anatomical Correction of Congenitally Corrected Transposition of Great Arteries (IDD) with Pulmonary Stenosis, Ventricular Septal Defect, Situs Inversus, and Levocardia

Objectives: Anatomical correction of congenitally corrected transposition of great arteries (ccTGA) with pulmonary stenosis (PS), malalignment ventricular septal defect (VSD) in situs inversus and levocardia presents significant surgical challenge. Reoperations due to recurrent obstruction of both outflow tracts following the Senning-Rastelli operation are serious long-term issues. A novel approach using truncal half turn and Senning operation was used to prevent these known late complications.

Anomalous origin of the right coronary artery from the pulmonary artery. Two case reports

Anomalous origin of the right coronary artery originating from the pulmonary trunk (ARCAPA) is a rare but potentially fatal anomaly. We are presenting two cases of ARCAPA and reviewing the main previous published data on this lesion. The first patient presented at the age of 5 months with respiratory distress and severe chest infection. He was found to have heart murmur and cardiomegaly on chest X ray. Echocardiographic and angiographic data confirmed an ARCAPA associated to a large malalignment ventricular septal defect and distal pulmonary artery aneurysms. He underwent surgical closure of the ventricular septal defect and reimplantation of the ARCAPA on the aorta with good result. The second case is an 11 year old male patient, complaining of dizziness and chest pain on exertion. Echocardiographic and angiographic data confirmed ARCAPA associated to a valvular pulmonary stenosis. He was operated on successfully. He got also direct reimplantation of the anomalous coronary artery on the aorta and a pulmonary valve commissurotomy. An anomalous origin of the right coronary artery is a rare condition but may lead to myocardial ischemia and sudden death. Diagnosis is mainly made by echocardiography and confirmed by conventional coronary arteriography. Operative correction is the appropriate treatment for an anomalous coronary artery arising from the pulmonary trunk.

Adult congenital heart disease and pulmonary hypertension: management of a complex case

To the Editor: Patients with congenital heart disease (CHD) are at increased risk of developing pulmonary arterial hypertension (PAH). These patients have a particularly poor prognosis if untreated when compared with CHD patients without PAH [1]. This case study demonstrates how a complete work-up and continuous follow-up in expert centres is required to ensure that PAH-CHD patients are managed appropriately. A female patient initially presented at a local community hospital at 3 yrs of age when she had failed to thrive. Echocardiography showed the presence of a moderately sized ventricular septal defect (VSD) and pulmonary stenosis. She underwent surgery for pulmonary stenosis with right ventricle outflow tract (RVOT) augmentation and valvectomy. However, the VSD was left open for reasons that were not specified in the patient's notes. Following this procedure, the patient moved out of the area and was lost to follow-up. After 16 yrs, in 2006 and at the age of 19 yrs, she presented to a local cardiologist to enquire whether she could become pregnant. On assessment, she was found to have mild dyspnoea in World Health Organization functional class II/III, oxygen saturation of 91% on room air, loud P2 heart sounds, II/VI holosystolic murmur at the left sternal border, normal jugular venous pressure and no evidence of oedema. Transthoracic echocardiography (TTE) showed a mildly enlarged right ventricle with normal systolic function, a moderately sized malaligned VSD with left-to-right (systemic-to-pulmonary) shunting, and normal left ventricle size and systolic function. Based on these findings, the patient was referred for immediate VSD closure. At this time, pregnancy was not ruled out. The patient was subsequently referred to an adult CHD/pulmonary hypertension (PH) expert centre for further evaluation. At the adult CHD/PH evaluation, the patient complained of dyspnoea with hill walking, climbing stairs or when carrying heavy loads, …

Non-Destructive, High-Resolution 3-Dimensional Visualization of a Cardiac Defect in the Chick Embryo Resembling Complex Heart Defect in Humans Using Micro-Computed Tomography

Nondestructive, high-resolution 3-dimensional (3D) imaging of the embryonic heart remains a challenge in cardiovascular development research. In the past, several imaging techniques (eg, magnetic resonance microscopy, optical coherence tomography) were tested for their suitability to visualize the 3D morphology of embryonic hearts. Most of these imaging tools have their drawbacks with respect to resolution and depth penetration. Here we present, to the best of our knowledge, the first high-resolution 3D images of normal and malformed embryonic chick hearts at the 10 μm level generated by microcomputed tomography (micro-CT) examination of critical point-dried heart specimens. Cardiac anatomy is demonstrated in great details with respect to myocardial fiber arrangement and trabeculations as well as atrioventricular (AV) and semilunar valves. Positions of great vessels with associated ventricular septal defects are visualized in high quality in malformed hearts. Figure 1A shows the normal 4-chambered heart of a day 9 chick embryo with correct positioning of the heart chambers and great vessels. Figure 1B depicts an image of a malformed embryonic chick heart in which both great arteries are significantly shifted toward the right side with extreme dextroposition of the aorta, and both great arteries arise from the right ventricle (double outlet right ventricle [DORV]). Note also that both atrial appendages are located to the left of the great arteries (left juxtaposition of atrial appendages [LJAA]). The cardiac defect that we present here in the chick embryo resembles a rare complex heart defect known in humans, as depicted in a classic drawing by Frank Netter, MD, in the Netter Collection of Medical Illustrations – Heart (Figure 1C, reproduced with permission from netterimages.com). Figure 1. Photographs of day 9 embryonic chick hearts (A and B, respectively) and a drawing of a malformed human …

Restrictive ventricular septal defect and critical subaortic stenosis in tetralogy of Fallot

Tetralogy of Fallot is characterized by a non-restrictive malalignment ventricular septal defect (VSD), an overriding aorta and right ventricular outflow tract obstruction resulting from anterior deviation of the infundibular septum. Due to the large VSD, systolic pressures are equal in both ventricles. In rare cases, redundant tricuspid valve tissue may prolapse into the VSD resulting in restriction of the defect size and causing suprasystemic pressure in the right ventricle. Subaortic obstruction by prolapse of the redundant tricuspid tissue into the left ventricular outflow tract, although theoretically possible in this situation, has not been described yet in the literature. We report on a newborn with tetralogy of Fallot presenting with cyanosis and severe arterial hypotension a few hours after birth. Echocardiography revealed redundant hammock-like accessory tricuspid valve tissue almost completely occluding the originally large VSD. Suprasystemic pressure in the right ventricle resulted in protrusion of tricuspid valve tissue across the VSD and thereby caused severe left ventricular outflow tract obstruction. Emergency pulmonary balloon valvuloplasty performed for decompression of the right ventricle, reduced prolapse of tricuspid tissue into the left ventricular outflow tract and resulted in significant improvement of pulmonary and systemic blood flow. In tetralogy of Fallot, accessory tricuspid valve tissue may obstruct the VSD as well as the left ventricular outflow tract resulting in a life threatening condition in newborns shortly after birth.

Repaired tetralogy of Fallot in the adult: monitoring and management

Tetralogy of Fallot (ToF) is the most common cyanotic congenital heart disease (CHD). The history of surgical treatment of ToF is closely linked with the origins of cardiac surgery. Before...

Rapid Regression of Classic Clinical Signs of Cyanosis Accompanied by Disappearing Major Aortopulmonary Collaterals After Surgical Palliation Only in a 6-Year-Old Girl With Tetralogy of Fallot

Tetralogy of Fallot is still the most common cyanotic heart defect and occurs in ≈1 in 3600 newborns or in 3.5% of the newborns with congenital heart disease.1,2 Since the first efforts of surgical repair in the 1950s, operation techniques with right ventricular outflow tract augmentation (RVOTA) and closure of ventricular septal defects have improved continuously. Today, in countries providing a high standard of medical care, patients with uncorrected tetralogy of Fallot presenting classic clinical signs of cyanosis are rare. A 6-year-old girl from Afghanistan with tetralogy of Fallot, a large malalignment ventricular septal defect, and high-grade infundibular pulmonary stenosis together with a hypoplastic pulmonary system had undergone a left-sided modified Blalock-Taussig shunt in her home country (exact date of surgery could not be determined). No further interventions were performed until the girl was transferred to Germany at 6 years of age. The clinical signs of chronic cyanosis at this time consisted of an extreme clubbing of the digits, especially of the toes, bluish skin, and a deep central cyanosis as indicated by blue lips and a cyanosed tongue (Figure 1A, 1D, and 1G). The oxygen saturation, measured transcutanously, was ≈65%. The hemoglobin level was 20 g/dL. Figure 1. Images …

Membranous Septal Aneurysm: An Unusual Cause for Right Ventricular Outflow Tract Obstruction in a Malaligned Ventricular Septal Defect with Aortomitral Discontinuity (Double-Outlet Right Ventricle) Associated with Visceral Heterotaxy

The development of a septal aneurysm in the natural history of membranous ventricular septal defects usually makes the defect hemodynamically less significant. This report describes a case of severe right ventricular outflow obstruction produced by a membranous septal aneurysm in a patient who had an anterior malaligned ventricular septal defect with aortomitral discontinuity (double-outlet right ventricle). This patient did not have pulmonary stenosis other than the dynamic obstruction produced by the septal aneurysm. In this patient, the septal aneurysm produced both favorable and unfavorable hemodynamic effects. A reduction in the size of the ventricular septal defect produced a favorable effect, whereas a right ventricular outflow obstruction led to the unfavorable situation of right ventricular hypertension and hypertrophy. The large septal aneurysm in the presence of an already compromised right ventricular outflow tract related to an anteriorly malaligned septum resulted in severe obstruction.