Ostium Primum Research Articles

Minimally invasive pediatric surgery in uncomplicated congenital heart disease

We aimed to highlight the use of a minimally invasive approach in uncomplicated congenital heart surgery. We investigated retrospectively 32 children below 10 years of age who underwent elective closure of ostium secundum type (n = 27), sinus venosus type (n = 4) and ostium primum type (n = 1) atrial septal defects through a limited skin incision and partial lower sternotomy between August 2001 and December 2008. All patients had cannulation through the same incision for cardiopulmonary bypass. A pericardial patch was used to close the defect in 8 patients and direct suturing in 24. The mean time from the skin incision to cannulation was 56 ± 23 min. Total bypass time was 27 ± 12 min, and crossclamp time was 15 ± 8 min. Mean length of hospital stay was 4 ± 2 days. We did not encounter any complications or mortality. A minimally invasive approach, consisting of a limited skin incision and partial lower sternotomy, is a safe, reliable, and cosmetically advantageous method in uncomplicated congenital heart disease surgery, which can be performed widely, and may replace the standard approach without increasing mortality and morbidity.

Incidental post-surgical pseudoaneurysm of the left ventricle: an unexpected finding

A 48-year-old female, with a history of ostium primum atrial septal defect repair, had a closure of a residual shunt and repair of a regurgitant left atrioventricular valve complicated by excessive intraoperative bleeding from an unknown source, which was successfully managed conservatively. Post-operative transthoracic echocardiograms (TTE) were unremarkable. The follow-up cardiovascular magnetic resonance (CMR) scan 4 years later, demonstrated an incidental distal anterolateral wall, thin-walled and akinetic outpouching, communicating with the left ventricle through a narrow neck. Following …

Partial Atrioventricular Septal Defect Detected After Transcatheter Intervention

A 74-year-old woman was referred to our hospital for surgery. She was suffering from mild mitral regurgitation (MR) and residual left-to-right shunt after closure of an atrial septal defect with the Amplatzer septal occluder 1 year earlier. Preoperative echocardiography showed grade II MR and revealed an ostium primum defect of 9 mm in diameter (Fig 1A, between two arrows; AML anterior mitral leaflet; LA left atrium; RA right atrium, RV right ventricle). Furthermore, intraoperative three-dimensional echocardiography showed a cleft of the anterior mitral leaflet (Fig 1B, arrow).

Expression of the BMP Receptor Alk3 in the Second Heart Field Is Essential for Development of the Dorsal Mesenchymal Protrusion and Atrioventricular Septation

The dorsal mesenchymal protrusion (DMP) is a prong of mesenchyme derived from the second heart field (SHF) located at the venous pole of the developing heart. Recent studies have shown that perturbation of its development is associated with the pathogenesis of atrioventricular (AV) septal defect. Although the importance of the DMP to AV septation is now established, the molecular and cellular mechanisms underlying its development are far from fully understood. Prior studies have demonstrated that bone morphogenetic protein (BMP) signaling is essential for proper formation of the AV endocardial cushions and the cardiac outflow tract. A role for BMP signaling in regulation of DMP development remained to be elucidated. To determine the role of BMP signaling in DMP development. Conditional deletion of the BMP receptor Alk3 from venous pole SHF cells leads to impaired formation of the DMP and a completely penetrant phenotype of ostium primum defect, a hallmark feature of AV septal defects. Analysis of mutants revealed decreased proliferative index of SHF cells and, consequently, reduced number of SHF cells at the cardiac venous pole. In contrast, volume and expression of markers associated with proliferation and active BMP/transforming growth factor β signaling were not significantly altered in the AV cushions of SHF-Alk3 mutants. BMP signaling is required for expansion of the SHF-derived DMP progenitor population at the cardiac venous pole. Perturbation of Alk3-mediated BMP signaling from the SHF results in impaired development of the DMP and ostium primum defects.

Isolated cleft of the anteroir leaflet of the mitral valve: Case report in a nine month old boy at Delta State University Teaching Hospital, Oghara, Niger Delta, Nigeria

Isolated cleft of the anterior leaflet of the mitral valve is the occurence of mitral cleft without ostium primum or ostio seccundum defect and it is rare. We present a nine month old boy who presented in our clinic with a two month history of fast breathing, cough, and loss of weight. He was found not to be cyanosed but pale, tachypnoeaic, dyspnoeaic with basal crepitations. Cadiovascular system examination showed that he had a precordial bulge with tachycardia, cardiomegaly and a holosystolic murmur maximum at the apex. There was tender hepatomegaly. He was diagnosed as having congestive cardiac failure with broncho pneumonia secondary to an acyanotic congenital heart disease. Plain chest X-ray confirmed the cardiomegaly and bronchopneumonia while an electrocardiography showed bilateral atrial enlargement with right ventricular hypertrophy. The cardiac failure was treated but he represented with recurrent heart failure upto three times within six months. An echocardiogram done on the third admission showed an isolated cleft of the anterior leaflet of the mitral valve and he has been referred for surgery. Isolated cleft of the mitral valve commonly presents with mitral incompetence and eventual heart failure as in our patient. Early surgical intervention is adviced as the width of the cleft tend to increase as the child grows leading to worsening of the mitral insufficiency. Key words: Cleft, anterior leaflet, mitral valve.

An isolated cleft of the anterior leaflet of mitral valve in an infant: a case report

Background : Isolated cleft of the anterior leaflet of the mitral valve is the occurrence of mitral cleft without ostium primum or ostium secundum defect and it is rare. It results from various degrees of failure of fusion of early embroyonic atrioventricular endocardial cushions and occurs more in children with trisomy 21. It commonly presents with mitral incompetence which worsens as the child grows. Aim : To document a case of congenital isolated cleft of the anterior leaflet of the mitrial valve in an infant. Findings : A nine month old boy presented in our clinic with a two month history of fast breathing, cough, and loss of weight. He was found not to be cyanosed but pale, tachypnoeic, dyspnoeic with basal crepitations. Cadiovascular system examination revealed he had a precordial bulge with tachycardia, cardiomegaly and a holosystolic murmur maximum at the apex. There was tender hepatomegaly. He was diagnosed as having congestive cardiac failure with broncho- pneumonia secondary to an acyanotic congenital heart disease. Plain chest X-ray confirmed the cardiomegaly and bronchopneumonia while an electrocardiography showed bilateral atrial enlargement with right ventricular hypertrophy. The cardiac failure was treated but he represented with recurrent heart failure upto three times within six months. An echocardiogram done on the third admission showed an isolated cleft of the anterior leaflet of the mitral valve and he has been referred for surgery. Conclusion : Isolated cleft of the mitral valve commonly presents with mitral incompetence and eventual heart failure as in our patient. Early surgical intervention is adviced as the width of the cleft tend to increase as the child grows leading to worsening of the mitral insufficiency. The definitive treatment is direct closure of the cleft. The presentation also highlights the challenges of medical practice in resource-poor countries. Keywords : Congenital , heart, isolated cleft, mitral valve, ostium primum, ostium secundum

Surgical Experience of a Partial Atrioventricular Septal Defect in an Elderly Patient: A Case Report

The following paper describes patch closure (bovine pericardial sheet) of an ostium primum atrial septal defect and mitral valve repair (sutured mitral valve cleft + autologous pericardial annuloplasty + Alfieri’s method) in a 50-year-old man. He had been perfectly well until he was brought to the emergency room because of acute heart failure. Chest radiography revealed right-side heart enlargement. Electrocardiography indicated atrial flutter. Echocardiography revealed a large ostium primum atrial septal defect and moderate mitral regurgitation. The pulmonary-to-systemic blood flow ratio was 3.24. First, cardiologists performed catheter ablation of the cavotricuspid isthmus for atrial flutter. We performed patch closure of an ostium primum atrial septal defect and mitral valve repair after the patient’s heart failure was under control. The patient was discharged 13 days postoperatively in a satisfactory condition without any critical complications.

Clinical experiences on correction of congenital heart diseases with robotic technology: a report of 160 cases

To summarize the clinical experiences on correction of congenital heart disease with robotic technology at a single center. Between January 2007 and May 2012, this retrospective study recruited 160 consecutive patients undergoing robotic surgery for congenital heart diseases. There were 74 males and 86 females with a median age of 35 years (range: 11-62). The procedures included secundum-type atrial septal defect repair (n = 130), ostium primum defect repair (n = 1), perimembranous ventricular septal defect repair (n = 21), mitral valve repair for anterior leaflet cleft (n = 7) and mitral valve repair plus left atrial myxoma resection (n = 1). Cardiopulmonary bypass graft was established through cannulation of right femoral artery, vein and right internal jugular vein under the guidance of transesophageal ultrasound. Myocardial protection was performed with cold blood cardioplegic solution or HTK solution and a transthoracic Chitwood clamp was used to occlude ascending aorta. Via three 8-mm ports and one 15-mm port in right chest, the microscopic instruments were manipulated to complete defect closure or mitral valve plasty, utilizing da Vinci S or da Vinci SI robotic system. Echocardiography was performed intraoperatively and at pre-discharge. Routine follow-ups were conducted. The clinical data of operating time, cardiopulmonary bypass time and follow-up examinations were retrospectively analyzed. All cases were treated successfully without a conversion into median sternotomy. No operative mortality or severe surgical complications were observed. Seventy-six cases of secundum-type atrial septal defect were completed on beating heart. The learning curves were noted for operating time of beating heart group and cross clamp time of arrest heart group. No residual shunt, malignant arrhythmia or mitral valve regurgitation was detected on intraoperative or postoperative echocardiography and during a median follow-up period of (29.1 ± 16.3) months. Robotic minimal access is technically feasible and it may be applied in selective patients with atrial septal defect, perimembranous ventricular septal defect and mitral valve cleft.

Polydactyly and ostium primum type atrial septal defect: Ellis-van Creveld syndrome

Comorbid polydactyly and atrial septal defect (ASD) is a very rarely seen anomaly. Basic clinical findings of Ellis-van Creveld syndrome include muscular skeletal disorders in the hands in 100% of cases, additional symmetrical postaxial polydactyly in the feet in 10-25% of cases, disproportionate dwarfism, a narrow thorax leading to respiratory problems, ectodermic dysplasia in the skin, nails, and teeth, a high palate, malocclusion, micrognathia, and congenital heart diseases, especially ASDs, in almost half of the patients (50-60%). In this article, we report a case with polydactyly and primum type ASD along with the surgical treatment and outcomes.

Consensus on Timing of Intervention for Common Congenital Heart Diseases: Part I - Acyanotic Heart Defects

The purpose of this review/editorial is to discuss how and when to treat the most common acyanotic congenital heart defects (CHD); the discussion of cyanotic heart defects will be presented in a subsequent editorial. By and large, the indications and timing of intervention are decided by the severity of the lesion. Balloon pulmonary valvuloplasty is the treatment of choice for valvar pulmonary stenosis and the indication for intervention is peak-to-peak systolic pressure gradient >50 mmHg across the pulmonary valve. For aortic valve stenosis, balloon aortic valvuloplasty appears to be the first therapeutic procedure of choice; the indications for balloon dilatation of aortic valve are peak-to-peak systolic pressure gradient across the aortic valve in excess of 70 mmHg irrespective of the symptoms or a gradient ≥ 50 mmHg with either symptoms or electrocardiographic ST-T wave changes indicative of myocardial perfusion abnormality. The indications for intervention in coarctation of the aorta are significant hypertension and/or congestive heart failure along with a pressure gradient in excess of 20 mmHg across the coarctation; the type of intervention varies with age at presentation and the anatomy of coarctation: surgical intervention for neonates and young infants, balloon angioplasty for discrete native coarctation in children, and stents in adolescents and adults. Long segment coarctations or those associated with hypoplasia of the isthmus or transverse aortic arch require surgical treatment in younger children and stents in adolescents and adults. For post-surgical aortic recoarctation, balloon angioplasty in young children and stents in adolescents and adults are treatment options. Transcatheter closure methods are currently preferred for ostium secundum atrial septal defects (ASDs); the indications for occlusion are right ventricular volume overload by echocardiogram. Ostium primum, sinus venosus and coronary sinus ASDs require surgical closure. For all ASDs elective closure around age 4 to 5 y is recommended or as and when detected beyond that age. For the more common perimembraneous ventricular septal defects (VSDs) of large size, surgical closure should be performed prior to 6 to 12 mo of age. Muscular VSDs may be closed with devices. Patent ductus arteriosus (PDA) may be closed with Amplatzer Duct Occluder if they are moderate to large and Gianturco coils if they are small. Surgical and video-thoracoscopic closure are the available options at some centers. In the presence of pulmonary hypertension appropriate testing to determine suitability for closure should be undertaken. The treatment of acyanotic CHD with currently available medical, transcatheter and surgical methods is feasible, safe and effective and should be performed at an appropriate age in order to prevent damage to cardiovascular structures.

Specific association of missense mutations in CRELD1 with cardiac atrioventricular septal defects in heterotaxy syndrome

Specific association of missense mutations in <i>CRELD1</i> with cardiac atrioventricular septal defects in heterotaxy syndrome

Closure of Interatrial Defects by Immediate-Release Patch

The Immediate-Release Patch is the newest version of the Transcatheter Patch, which is a bioabsorbable device for the closure of cardiac defects. Closure of 12 atrial septal defects and 1 fenestration was attempted using this device. Of the 13 devices used, 12 were implanted successfully (including that for the fenestration), whereas 1 device moved from the original position and was retrieved percutaneously. No other major adverse events occurred. After a median follow-up period of 11 months, trivial (≤ 2-mm) residual shunts remained in two patients, and a significant residual defect (7 mm) remained in 1 patient. The major advantages of this device include its wirelessness, its bioabsorbablity, potential application for defects up to 30 mm using only three sizes, its easier retrieval, and its ability to occlude defects with a deficient rim and some ostium primum and sinus venosus type defects. In contrast to the metal devices, it is bulkier and has a different application method requiring operator familiarity, and it sometimes leaves residual shunts.

Tetralogy of Fallot with Holt-Oram syndrome

Holt-Oram syndrome (HOS) is characterised by mild to severe congenital cardiac defects and skeletal abnormalities of the upper limb. This syndrome is also referred to as Hand-Heart syndrome. The most common cardiac disorder is an ostium secundum detected an atrial septal defect (ASD), followed by ventricular septal defect (VSD) and ostium primum ASD. We report a case of HOS with tetralogy of Fallot (TOF). This association is very rare and is hardly reported in the literature.

Associated double-orifice mitral and tricuspid valves without ostium primum defect

The simultaneous existence of double orifice right and left atrioventricular valves in the absence of ostium primum defects is rare and scarcely reported. We present the case of a 20-month old boy diagnosed with tetralogy of Fallot with pulmonary atresia who was found to have associated double-orifice mitral and tricuspid valves.

Double-orifice mitral valve: a rare congenital anomaly

A 39-year old male presented with dyspnoea, NYHA class II. The diagnosis of an ostium primum atrial septal defect (ASD) with a double-orifice mitral valve was established by transthoracic echocardiography (TTE) (Fig. 1A) as well as two-dimensional (2D) and 3D transoesophageal echocardiography (TEE) (Figs 1B and 2A). Intraoperative findings are revealed in Fig. 2B. Successful closure of the primum ASD was performed.

Real-Time 3-Dimensional Transesophageal Echocardiography of the Atrioventricular Septal Defect

Being able to view the morphological details of the heart malformation in any patient with the diagnosis of atrioventricular septal defect (AVSD) is invaluable in surgical planning, especially in the current era of modified 1-patch versus 2-patch techniques. Real-time 3-dimensional transesophageal echocardiography (RT 3D TEE) is a recently developed technique that allows real-time high-quality images akin to viewing the heart specimen. We present the principal pathomorphological features of AVSD as visualized by RT 3D TEE. The hallmark of AVSD is a common atrioventricular junction guarded by a common atrioventricular valve.1 In the normal heart, when seen from atrial perspective, the aortic valve is wedged between the mitral and tricuspid valve. In AVSD, because of the common AV junction, the aortic valve is in an “unwedged” position (Figure 1). The valve consists of 5 leaflets: the superior and inferior bridging leaflets, each of which overrides the ventricular septum, a left mural leaflet, a right anterior, and a right inferior leaflet (Figure 2). Whether the bridging leaflets are joined together distinguishes the complete form from the partial form. In partial AVSD (also termed “ostium primum ASD”), a tongue of leaflet tissue connects the bridging leaflets and the undersides of these leaflets …

Cor triatriatum sinister with incomplete atrioventricular septal defect in a cat

An 11-month-old, 3 kg, female domestic shorthair cat was referred to evaluate cardiac structure and function. Echocardiography revealed the membrane dividing the left atrium into two chambers, a large defect in the lower part of the atrial septum, and turbulent blood flow from the distal left atrium into the right atrium. These findings suggested cor triatriatum sinister (CTS) with incomplete atrioventricular septal defect (AVSD). The cat was treated with medications for management of congestive heart failure. In the end, she died from right-sided heart failure 17 months after the initial presentation. At necropsy, a fibromuscular membrane with a round orifice in the left atrium and an ostium primum defect were confirmed, and the definitive diagnosis of CTS with incomplete AVSD was made. To our knowledge, this study presents the first case report of CTS with incomplete AVSD in a cat.

Surgical Repair of a Complete Endocardial Cushion Defect in a Dog

To describe surgical repair of a complete endocardial cushion defect (ECD) in a dog. Clinical report. A 5-month-old, 9.2 kg male Shetland sheepdog. Echocardiographic examination revealed an ostium primum atrial septal defect (ASD), an inlet ventricular septal defect (VSD), mitral regurgitation (MR) and tricuspid regurgitation (TR), and a complete ECD was diagnosed. Surgical correction was performed using cardiopulmonary bypass (CPB) via right atriotomy. A polytetrafluoroethylene (PTFE) patch was secured along the margin of the inlet VSD using simple continuous suture, then the cleft in the septal mitral leaflet was sutured. Similarly, the cleft in the septal tricuspid leaflets was sutured. To complete inlet VSD closure, the VSD patch was secured to these sutured leaflets by simple continuous suture. Another PTFE patch was used to close the ostium primum ASD. After surgery, MR, TR, and interventricular shunting were decreased. The dog was alive 6 years and 5 months after the surgery with no evidence of an interventricular shunt, TR, or other clinical signs. Complete ECD in a dog was corrected using a 2-patch technique under CPB.

Cirugía mínimamente invasiva en niños. Corrección de la comunicación interauricular por vía axilar y submamaria

Introduction and objectives Minimally invasive approaches in less-complex cardiac procedures can avoid unpleasant cosmetic results. Moreover, surgery can be scheduled in younger patients. In previous papers, we compared submammary and midline sternotomy. We present our initial experience with an axillary, compared to submammary, approach to repair atrial septal defects under extracorporeal circulation. Methods 20 patients are included: 10 in the submammary group (7 ostium secundum, 2 sinus venosus, 1 ostium primum) and 10 in the axillary group (7 ostium secundum, 3 sinus venosus). Mean age and weight are 6.4 ± 3.62 years (range 3-13) and 23.5 ± 8.74 Kg (range 12-38) in the submammary group, and 5.5 ± 2.04 years (range 3-9) and 19.7 ± 5.88 Kg (range 14-29) in the axillary one, respectively. Muscles are spared (pectoralis in submammary and latissimus in axillary). The whole procedure (cannulation and correction) is performed through a single incision, with no side ports. Results No residual defects were found at discharge. Surgical approach maneuvers are more cumbersome through the axillary than the submammary approach. In a peer comparison, extracorporeal circulation and cross-clamp time were similar in both groups ( P > .05). Conclusions 1. Axillary approach is as safe as submammary access in selected patients and for defects approached through the atrium. 2. Cosmetic result is excellent. Full English text available from: www.revespcardiol.org

Minimally Invasive Pediatric Cardiac Surgery. Atrial Septal Defect Closure Through Axillary and Submammary Approaches

Introduction and objectives Minimally invasive approaches in less-complex cardiac procedures can avoid unpleasant cosmetic results. Moreover, surgery can be scheduled in younger patients. In previous papers, we compared submammary and midline sternotomy. We present our initial experience with an axillary, compared to submammary, approach to repair atrial septal defects under extracorporeal circulation. Methods 20 patients are included: 10 in the submammary group (7 ostium secundum, 2 sinus venosus, 1 ostium primum) and 10 in the axillary group (7 ostium secundum, 3 sinus venosus). Mean age and weight are 6.4 ± 3.62 years (range 3-13) and 23.5 ± 8.74 Kg (range 12-38) in the submammary group, and 5.5 ± 2.04 years (range 3-9) and 19.7 ± 5.88 Kg (range 14-29) in the axillary one, respectively. Muscles are spared (pectoralis in submammary and latissimus in axillary). The whole procedure (cannulation and correction) is performed through a single incision, with no side ports. Results No residual defects were found at discharge. Surgical approach maneuvers are more cumbersome through the axillary than the submammary approach. In a peer comparison, extracorporeal circulation and cross-clamp time were similar in both groups ( P>.05). Conclusions 1. Axillary approach is as safe as submammary access in selected patients and for defects approached through the atrium. 2. Cosmetic result is excellent.