Restrictive Atrial Septal Defect Research Articles

Atrial Septal Defect Stent Compression During Resuscitation of an Infant With Hypoplastic Left Heart Syndrome.

A newborn with hypoplastic left heart syndrome underwent a Norwood procedure with a Sano shunt. A month later, he had an atrial septal defect (ASD) stent placed due to ASD size and flow restriction. Three weeks later, he had sudden bradycardia and cardiac arrest in the intensive care unit. He was resuscitated and cannulated for extracorporeal membrane oxygenation (ECMO) through a right neck approach. An attempt to wean him from ECMO a week later failed due to hypoxia. Echocardiography showed ASD flow restriction and a chest x-ray showed flattening of the left atrial side of the ASD stent from chest compression resulting in a restrictive ASD. The patient underwent surgical removal of the ASD stent with ASD enlargement and was successfully weaned from ECMO after surgery.

Echocardiographic imaging of neonatal transposition of great arteries: Rare case report and review of literature

Transposition of the great arteries (TGA) is a congenital conotruncal abnormality characterized by discordant connections between the ventricles and great arteries, with the aorta originating from the right ventricle (RV), and the pulmonary artery (PA) originating from the left ventricle (LV). The two main types of TGA are complete transposition or dextro-transposition of the great arteries (D-TGA), commonly referred to as D-loop, and congenitally corrected transposition (CCTGA), commonly referred to as L-loop or L-TGA. In D-TGA, the connections between the ventricles and atria are concordant, whereas in CCTGA they are discordant, with the left atrium connected to the RV, and the right atrium connected to the LV. Imaging plays an important role in the evaluation of TGA, both before and after surgery, for helping define the anatomy, quantify hemodynamics, and evaluate complications. Transthoracic echocardiography is the first-line imaging modality for pre-surgical planning in children with TGA. MRI provides comprehensive morphologic and functional information, particularly in adults after surgery. CT is performed when MRI is contraindicated or expected to generate artifacts. We are reporting an extremely rare and unique case of 25-day old male neonate, suffering from TGA with a side to side alignment of great arteries, accompanied by a moderate sized restrictive atrial septal defect (ASD) and an ascending aortic aneurysm (AAA).

Staged left ventricular recruitment facilitated by Sano conduit upsizing

Staged left ventricular recruitment facilitated by Sano conduit upsizing

Clinical and haemodynamic variables associated with intensive care unit length of stay and early adverse outcomes after the Norwood procedure.

This study was performed to determine the clinical and haemodynamic variables associated with early adverse outcomes after the neonatal Norwood procedure. Patients who underwent the neonatal Norwood procedure between 2001 and 2019 were included. The patient diagnosis, morphological characteristics and haemodynamic parameters were analysed to identify factors associated with length of stay (LOS) in the intensive care unit (ICU) and mortality during the stay. A total of 322 patients were depicted. The median age and weight at the Norwood procedure were 9 days and 3.2 kg, respectively. Certain morphological and preoperative parameters, such as birth weight below 2.5 kg, restrictive atrial septal defect, extracardiac anomalies and the diameter of the ascending aorta, were found to be associated with the LOS in the ICU. Analysis using early postoperative haemodynamic variables revealed that systolic arterial pressure, diastolic arterial pressure, serum lactate levels and reduced ventricular function at 2 days postoperatively were associated with the LOS in the ICU. Birth weight <2.5 kg (P = 0.010), a restrictive atrial septal defect (P = 0.001) and smaller ascending aorta (P = 0.039) were associated with death in the ICU. Reduced ventricular function, lower systolic aortic pressure and higher lactate levels at various time points (P < 0.05) were also associated with ICU deaths. The LOS in the ICU was significantly associated with late mortality (P < 0.001, Hazard Ratio (HR) = 1.015). The LOS in the ICU after the Norwood procedure was predicted by early postoperative haemodynamic variables, suggesting that good early postoperative haemodynamics determine early recovery. A prolonged stay in the ICU after the Norwood procedure was associated with late mortality.

Safety of Transseptal Puncture for Access to the Left Atrium in Infants and Children

Transseptal puncture (TSP) is a standard procedure to obtain access to the left heart. However, data on TSP in infants and children particularly with congenital heart defects (CHD) is sparse. Safety and efficacy of TSP in infants and children < 18 years with normal cardiac anatomy and with CHD were assessed. 327 TSP were performed in a total of 300 individuals < 18 years from 10/2002 to 09/2018 in our tertiary pediatric referral center. Median age at TSP was 11.9 years (IQR 7.8–15; range: first day of life to 17.9 years). 13 subjects were < 1 year. Median body weight was 43.8 kg (IQR 26.9–60; range: 1.8–121 kg). CHD was present in 28/327 (8.6%) procedures. TSP could be successfully performed in 323/327 (98.8%) procedures and was abandoned in 4 procedures due to imminent or incurred complications. Major complications occurred in 4 patients. 3 of these 4 subjects were ≤ 1 year of age and required TSP for enlargement of a restrictive atrial septal defect in complex CHD. Two of these babies deceased within 48 h after TSP attempt. The third baby needed urgent surgery in the cath lab. Pericardial effusion requiring drainage was noted in the forth patient (> 1 year) who was discharged well later. Minor complications emerged in 5 patients. The youngest of these individuals (0.3 years, 5.8 kg) developed small pericardial effusion after anterograde ballon valvuloplasty for critical aortic stenosis. The remaining 4/5 patients developed small pericardial effusion after ablation of a left-sided accessory atrioventricular pathway (6.1–12.2 years, 15.6–34.0 kg). TSP for access to the left heart was safe and effective in children and adolescents > 1 year of age. However, TSP was a high-risk procedure in small infants with a restrictive interatrial septum with need for enlargement of interatrial communication.

Bail-out wire stenting of restrictive atrial septal defect in cardiac total anomalous pulmonary venous connection.

Bail-out wire stenting of restrictive atrial septal defect in cardiac total anomalous pulmonary venous connection.

Hemodynamic assessment of atrial septal defects.

Atrial septal defect (ASD) is one of the most common congenital cardiac anomalies. ASD can present as an isolated lesion in an otherwise normal heart or in association with other congenital heart conditions. Regardless of the type of ASD, the direction and degree of shunting across the communication is mainly determined by the difference in compliance between the right and left ventricle. Hemodynamics in children is characterized by left-to-right shunting, dilated right heart structures and normal pulmonary artery pressures (PAP). Patients diagnosed at adult age often present with complications related to long-standing volume overload such as pulmonary artery hypertension and right and left ventricular dysfunction. Diagnostic catheterization is usually not indicated unless there is suggestion of pulmonary hypertension on echocardiography. In older patients and/or in those with ventricular dysfunction, measurement of left heart pressures during temporary balloon occlusion is recommended prior to device closure as it may not be tolerated. In ASD associated with other congenital malformations, shunting degree and direction will depend upon underlying condition. Restrictive ASD can result in significant hemodynamic compromise in neonates with conditions such as hypoplastic left heart syndrome (HLHS) and transposition of the great arteries (TGA). In most cases, hemodynamics can be estimated with echocardiography only.

Prolonged PR Interval at Birth Predicting the High Occurrence of Fatal Atrioventricular Block in Hypoplastic Left Heart Syndrome.

Infants with hypoplastic left heart syndrome (HLHS) are at high mortality especially when they are associated with bradyarrhythmias. However, the risk factor of developing high-grade atrioventricular block (HAVB) is still unclear. Seventy-three patients with HLHS in our institutions from 2002 to 2011 were enrolled. The survival rate was assessed by the anatomical types, treatments, occurrence of HAVB, severe tricuspid regurgitation (TR), and restrictive atrial septal defect (ASD) along with electrocardiogram findings at birth. There were 23 (32%) cardiogenic and 7 (10%) non-cardiogenic deaths. The occurrence rate of HAVB but not severe TR or restrictive ASD was higher in 30 deceased patients than in 43 survived patients [7 (23%) vs. 1 (2.3%), p = 0.0038]. The overall mortality rate was higher in patients with HAVB than in those without it (p = 0.0002). Of 7 deceased patients with HAVB, 6 HAVB occurred within 10 days post-surgery, and 3 HAVB led to the early death. The mortality rate of patients with prolonged PR (≥ 0.15s) but not wide QRS (> 0.08s) or prolonged QTc (> 0.43s) at birth was higher than each without it (p = 0.0106). Multivariate analysis indicated that prolonged PR but no other variables was independently associated with the mortality (hazard ratio: 2.948, p = 0.0104). Prolonged PR at birth in HLHS infants predicts the development of fatal HAVB.

Left Atrium Mass in an Infant with Complex Cyanotic Congenital Heart Disease

The clinical and echocardiographic findings in a 3-month-old cyanotic infant with congestive cardiac failure are described with stress on segmental approach to diagnosis. Echocardiography reveals dextrocardia, situs inversus, d-loop ventricle, tricuspid atresia, transposition of great vessels, subpulmonic ventricular septal defect (VSD), d-malposed aorta, restrictive atrial septal defect (ASD), and large mass in high-pressure left atrium (LA). Systemic veins drain to left-sided morphological right atrium (RA) and pulmonary veins returned to the right-sided morphological LA. In the presence of tricuspid atresia, blood from the morphological LA has only one outlet into the morphological RA through a restrictive ASD resulting in a high-pressure left atrial chamber. Blood from the morphological RA drains into the left-sided morphological left ventricle. From the left ventricle through the subpulmonic VSD, a portion of blood is directed into the pulmonary artery (PA) by passing the hypoplastic morphological right ventricle (RV) as the PA overrides the VSD. Blood flows from the hypoplastic RV into the aorta. Besides, LA mass seen in this 3 month-old-baby in the given setting is an extremely rare finding and to my knowledge has not been reported in literature before.

Atrial septal defect morphology and stenting in hypoplastic left heart syndrome after hybrid palliation.

Complex morphologies were more frequently related to restrictiveness. Stenting technique has a crucial role, as the procedure carries a significant risk for stent migration. Effective treatment of restrictive atrial septal defect is related to a better outcome, as it leads to equalisation of survival between patients with and those without restrictive atrial septal defect.

An alternative technique of atrial septectomy during bidirectional superior cavopulmonary anastomosis.

An atrial septectomy is often required to create or enlarge a pre-existing restrictive atrial septal defect in patients with univentricular hearts undergoing the bidirectional superior cavopulmonary anastomosis. We describe an alternative surgical technique through the transected cardiac end of the superior vena cava without a right atriotomy successfully performed in 26 patients.

Off-Pump Atrial Septectomy for Infants With Restrictive Atrial Septal Defect

The management of some subsets of patients with an unsuccessful balloon atrial septostomy (BAS) and poor general condition is typically challenging. Our novel technique involves off-pump atrial septectomy using laparoscopic forceps guided by intraoperative direct pericardial echocardiography. The technique was first used in a case of tricuspid atresia in a patient in preshock and was then used in 2 more patients with hypoplastic left heart syndrome (HLHS) (age, 10-116 days; body weight, 2.4-3.7 kg). There were no operative complications of embolism or bleeding in our series. Our off-pump atrial septectomy technique is an effective approach in specific cases.

What Can Cause Pulmonary Vascular Disease in Functionally Single Ventricle?

Definition of “functionally single ventricle” includes a wide spectrum of anatomical variants such as double inlet left or right ventricle, mitral or tricuspid atresia, unbalanced atrioventricular canal, heterotaxy syndrome with one normally developed ventricle or other congenital heart defects with severe hypoplasia of one of the ventricles [2]. Anatomical and hemodynamic abnormalities in these congenital heart lesions are characterized by presence of a common ventricular chamber, where blood from the left and right atrium mixes and then enters the aorta and pulmonary artery. This can cause pulmonary hypervolemia and hypertension (in patients without pulmonary stenosis), difficulties with blood outflow (in patients with mitral atresia/stenosis combined with restrictive atrial septal defect), intake of highly oxygenated blood into the pulmonary artery (in patients with transposition of the great arteries) as well as systemic hypoxemia and polycythemia.

Incremental epidural anaesthesia for emergency caesarean section in a patient with ostium secundum atrial septal defect and severe pulmonary stenosis with right to left shunt.

Sir, Association of atrial septal defect (ASD) and pulmonary stenosis (PS) with bidirectional shunt in pregnancy is a very rare entity. Death can occur anytime during pregnancy or labour in these patients, precipitated by altered cardiovascular physiology of pregnancy.[1] A 25-year-old primi, an unbooked case with 38 weeks of gestation was posted for emergency caesarean section (CS) for cephalopelvic disproportion. In the second trimester, assessment of breathlessness (NYHA Grade III) with two-dimensional echocardiography revealed an ostium secundum type of ASD (8 mm × 10 mm) with predominantly right to left shunt (Qp: Qs - 0.85), severe PS (Maximum velocity [Vmax ]/peak pressure gradient [PGmax ] - 4.8 m/s/70.4 mm Hg), mild tricuspid regurgitation (Vmax /PGmax - 2.7 m/s/30.5 mm Hg), mild pulmonary regurgitation (Vmax /PGmax - 2.1 m/s/17.58 mm Hg), right ventricular hypertrophy (right ventricle diastolic wall thickness 6 mm) and normal biventricular function with ejection fraction of 60%. In the operation room, oxygen saturation (on room air) was 90-95%, pulse rate was 114/min regular and noninvasive blood pressure was 150/90 mmHg. The patient was premedicated with injection ranitidine (50 mg) and injection metoclopramide (10 mg) and colloid was started at 50 ml/h. Injection ceftriaxone 1 g was administered for antibiotic coverage. The initial central venous pressure was 13 cm H2O and was maintained between 12 and 14 cm H2O. Under aseptic precautions, an 18-gauge epidural catheter was inserted at L3 -L4 epidural interspace. A total volume of 20 ml local anaesthetic solution (13 ml of 2% lignocaine with adrenaline (5 μg/ml) and 1 ml of sodium bicarbonate and 1 ml (50 mg) of tramadol diluted with 5 ml saline) was administered through the epidural catheter in 5 ml increments for achieving a block height up to T4 dermatome. At the time of skin incision, injection ketamine (50 mg) and injection midazolam (1 mg) was administered in titrating doses. CS proceeded uneventfully, and a healthy male baby was delivered with Apgar score of 8 and 9 at 1 and 5 min, respectively. A bolus dose of 0.4 mg intravenous methylergometrine was administered slowly, and a slow infusion of oxytocin 10 units was started. The surgery lasted for about 60 min, and the blood loss was 500 ml. A total of 450 ml colloid and 100 ml oxytocin-normal saline infusion was given. Post-operative analgesia was provided with epidural top-up, whenever the patient complained of pain or visual analogue score was ≥4. At 48th h of follow-up, she had maximal scores for post-operative pain relief and overall satisfaction. She was discharged on the 7th post-operative day and referred to cardiology department for further management. The mortality in patients with ASD and severe PS with right to left shunt in pregnancy is unknown,[2] as many unsuccessful cases are not reported. In our case, although the patient was having reversal of shunt, she was asymptomatic and had no signs of chronic cyanosis. This may be due to hyperdynamic circulation of pregnancy producing increased pulmonary gradients and the restrictive ASD causing inadequate mixing of blood to produce clinical cyanosis.[3] Epidural anaesthesia (EA) was planned because of its slower onset and less and delayed haemodynamic variations compared to spinal and combined spinal-epidural technique.[4] General anaesthesia (GA) poses clear risks related to pulmonary vascular resistance and magnitude of the shunt. As the patient was in labour, we had no sufficient time for further cardiac evaluation. Pulse oximeter is used to assess the degree of right to left shunt during the peri-operative period.[5] Our principle was to maintain the cardiac output and to avoid the fall in systemic vascular resistance (SVR). We optimised the SVR based on mean arterial pressure to avoid the increase in shunt magnitude. Oxytocin bolus can cause direct vasodilatation and reduce SVR with a compensatory increase in heart rate and cardiac output in pregnant women. Use of methylergometrine lowered the use of vasoconstrictors[6] and added to haemodynamic stability. Colloids provide greater haemodynamic stability by remaining in the intravascular space for longer time and avoid volume overloading; this helps to decrease the magnitude of shunt and right ventricular failure.[7] The use of lower dose of lignocaine with adrenaline, bicarbonate and tramadol provided a more rapid onset and superior quality block to touch.[8] We did not institute any thromboprophylaxis measures; EA itself can reduce risk of perioperative venous stasis and embolism. To conclude, EA can be safely and effectively used for CS in pregnant patients having pulmonary vascular disease with reversal of shunts provided the SVR is maintained throughout the surgical procedure. Major haemodynamic complications and risk of embolism during GA can be avoided if EA is properly planned and administered. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.

Incremental epidural anaesthesia for emergency caesarean section in a patient with ostium secundum atrial septal defect and severe pulmonary stenosis with right to left shunt

Sir, Association of atrial septal defect (ASD) and pulmonary stenosis (PS) with bidirectional shunt in pregnancy is a very rare entity. Death can occur anytime during pregnancy or labour in these patients, precipitated by altered cardiovascular physiology of pregnancy.[1] A 25-year-old primi, an unbooked case with 38 weeks of gestation was posted for emergency caesarean section (CS) for cephalopelvic disproportion. In the second trimester, assessment of breathlessness (NYHA Grade III) with two-dimensional echocardiography revealed an ostium secundum type of ASD (8 mm × 10 mm) with predominantly right to left shunt (Qp: Qs - 0.85), severe PS (Maximum velocity [Vmax ]/peak pressure gradient [PGmax ] - 4.8 m/s/70.4 mm Hg), mild tricuspid regurgitation (Vmax /PGmax - 2.7 m/s/30.5 mm Hg), mild pulmonary regurgitation (Vmax /PGmax - 2.1 m/s/17.58 mm Hg), right ventricular hypertrophy (right ventricle diastolic wall thickness 6 mm) and normal biventricular function with ejection fraction of 60%. In the operation room, oxygen saturation (on room air) was 90-95%, pulse rate was 114/min regular and noninvasive blood pressure was 150/90 mmHg. The patient was premedicated with injection ranitidine (50 mg) and injection metoclopramide (10 mg) and colloid was started at 50 ml/h. Injection ceftriaxone 1 g was administered for antibiotic coverage. The initial central venous pressure was 13 cm H2O and was maintained between 12 and 14 cm H2O. Under aseptic precautions, an 18-gauge epidural catheter was inserted at L3 -L4 epidural interspace. A total volume of 20 ml local anaesthetic solution (13 ml of 2% lignocaine with adrenaline (5 μg/ml) and 1 ml of sodium bicarbonate and 1 ml (50 mg) of tramadol diluted with 5 ml saline) was administered through the epidural catheter in 5 ml increments for achieving a block height up to T4 dermatome. At the time of skin incision, injection ketamine (50 mg) and injection midazolam (1 mg) was administered in titrating doses. CS proceeded uneventfully, and a healthy male baby was delivered with Apgar score of 8 and 9 at 1 and 5 min, respectively. A bolus dose of 0.4 mg intravenous methylergometrine was administered slowly, and a slow infusion of oxytocin 10 units was started. The surgery lasted for about 60 min, and the blood loss was 500 ml. A total of 450 ml colloid and 100 ml oxytocin-normal saline infusion was given. Post-operative analgesia was provided with epidural top-up, whenever the patient complained of pain or visual analogue score was ≥4. At 48th h of follow-up, she had maximal scores for post-operative pain relief and overall satisfaction. She was discharged on the 7th post-operative day and referred to cardiology department for further management. The mortality in patients with ASD and severe PS with right to left shunt in pregnancy is unknown,[2] as many unsuccessful cases are not reported. In our case, although the patient was having reversal of shunt, she was asymptomatic and had no signs of chronic cyanosis. This may be due to hyperdynamic circulation of pregnancy producing increased pulmonary gradients and the restrictive ASD causing inadequate mixing of blood to produce clinical cyanosis.[3] Epidural anaesthesia (EA) was planned because of its slower onset and less and delayed haemodynamic variations compared to spinal and combined spinal-epidural technique.[4] General anaesthesia (GA) poses clear risks related to pulmonary vascular resistance and magnitude of the shunt. As the patient was in labour, we had no sufficient time for further cardiac evaluation. Pulse oximeter is used to assess the degree of right to left shunt during the peri-operative period.[5] Our principle was to maintain the cardiac output and to avoid the fall in systemic vascular resistance (SVR). We optimised the SVR based on mean arterial pressure to avoid the increase in shunt magnitude. Oxytocin bolus can cause direct vasodilatation and reduce SVR with a compensatory increase in heart rate and cardiac output in pregnant women. Use of methylergometrine lowered the use of vasoconstrictors[6] and added to haemodynamic stability. Colloids provide greater haemodynamic stability by remaining in the intravascular space for longer time and avoid volume overloading; this helps to decrease the magnitude of shunt and right ventricular failure.[7] The use of lower dose of lignocaine with adrenaline, bicarbonate and tramadol provided a more rapid onset and superior quality block to touch.[8] We did not institute any thromboprophylaxis measures; EA itself can reduce risk of perioperative venous stasis and embolism. To conclude, EA can be safely and effectively used for CS in pregnant patients having pulmonary vascular disease with reversal of shunts provided the SVR is maintained throughout the surgical procedure. Major haemodynamic complications and risk of embolism during GA can be avoided if EA is properly planned and administered. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.

Síndrome de hipoplasia de corazón izquierdo: experiencia de 10 años de un programa de etapificación quirúrgica

Hypoplastic left heart syndrome (HLHS) is a lethal congenital heart disease in 95% of non-treated patients. Surgical staging is the main form of treatment, consisting of a 3-stage approach, beginning with the Norwood operation. Long term survival of treated patients is unknown in our country. Objectives1) To review our experience in the management of all patients seen with HLHS between January 2000 and June 2012. 2) Identify risk factors for mortality. Patients and methodRetrospective analysis of a single institution experience with a cohort of patients with HLHS. Clinical, surgical, and follow-up records were reviewed. ResultsOf the 76 patients with HLHS, 9 had a restrictive atrial septal defect (ASD), and 8 had an ascending aorta ≤2mm. Of the 65 out of 76 patients that were treated, 77% had a Norwood operation with pulmonary blood flow supplied by a right ventricle to pulmonary artery conduit, 17% had a Norwood with a Blalock-Taussig shunt, and 6% other surgical procedure. Surgical mortality at the first stage was 23%, and for Norwood operation 21.3%. For the period between 2000-2005, surgical mortality at the first stage was 36%, and between 2005-2010, 15% (P=.05). Actuarial survival was 64% at one year, and 57% at 5years. Using a multivariate analysis, a restrictive ASD and a diminutive aorta were high risk factors for mortality. ConclusionsOur immediate and long term outcome for staged surgical management of HLHS is similar to that reported by large centres. There is an improvement in surgical mortality in the second half of our experience. Risk factors for mortality are also identified.

Restrictive Atrial Septum Defect Becomes a Risk Factor for Norwood Palliation of Hypoplastic Left Heart Syndrome Only When It Is Combined with Mitral or Aortic Atresia

Restrictive atrial septal defect (ASD) is described as risk factor for Norwood procedure because of elevated pulmonary resistance. We hypothesized that it invariably could not cause pulmonary hypertension, unless it was combined with mitral valve or aortic valve atresia. We investigated how restrictive ASD influenced survival of patients with hypoplastic left heart syndrome (HLHS) who underwent Norwood operation. A total of 118 HLHS patients who underwent surgery between January 2005 and December 2012 were grouped into three groups. Group 1 included 31 patients with restrictive ASD combined with mitral or aortic atresia; Group 2 composed of 12 patients with restrictive ASD and mitral and aortic stenosis; Group 3 (n = 75) had no ASD restriction. Survival was determined for each group. Multivariate analysis was conducted to test risk factors for mortality. Mean follow-up was 26.3 ± 24.1 months. Survival was 78.7% ± 4.2% at 30-month interval and onward after Norwood procedure for the whole cohort; it was 43.8% ± 10.0%, 91.7% ± 8.0%, and 77.3% ± 5.0% for Group 1, 2, and 3, respectively. The difference was significant between Group 1 and Group 2 and 3: p < 0.001. Survival was similar for Group 2 and Group 3: p = 0.45. Combination of restrictive ASD and mitral or aortic atresia was found to be the sole risk factor for early and late mortality (odds ratio: 3.5, 95% confidence interval: 1.8-7.1, p < 0.001). Restrictive ASD only affects survival of HLHS patients following Norwood procedure if it is associated with mitral or aortic atresia.

Is Restrictive Atrial Septal Defect a Risk in Partial Anomalous Pulmonary Venous Drainage Repair?

The creation or enlargement of an atrial septal defect (ASD) in partial anomalous pulmonary venous drainage (PAPVD) repair may pose a risk of postoperative pulmonary vein stenosis (PVS), superior vena cava stenosis (SVCS), and atrial rhythm disturbances. 155 children who underwent repair of right PAPVD between 1990 and 2010 were reviewed. PVS and SVCS were defined by mean gradients on echocardiography: mild=3 to 5 mm Hg; severe=6 mm Hg or higher. Postoperative cardiac rhythms were categorized as sinus, transient nonsinus, and persistent nonsinus rhythms. Outcomes were compared between patients who underwent the creation or superior enlargement of an ASD (group A) and those who did not (group B). There was no early or late death. Freedom from any PVS at 15 years after operation was lower in group A than in group B (76.1% vs 96.5%, p=0.002), and no differences were found in freedom from severe PVS (p=0.103), any SVCS (p=0.419), or severe SVCS (p=0.373). Group A patients had more PVS-related reoperations (p=0.022). Nineteen patients had nonsinus rhythm, and 4 patients experienced first-degree atrioventricular block, but no significant difference was found between the groups. Cox regression revealed the creation or superior enlargement of an ASD as a predictor for postoperative PVS (p=0.032). A case-match analysis confirmed a higher risk of PVS in patients with the creation or superior enlargement of an ASD (p=0.018). Late outcomes after repair of PAPVD are excellent. The subgroup that requires creation or superior enlargement of an ASD in repair of a right PAPVD is at a higher risk of late PVS and a subsequent increase in PVS-related reoperation. The presence of restrictive ASD did not increase SVCS, sinus node, or atrial conduction dysfunction.

Percutaneous transhepatic stenting of a restrictive atrial septal communication in hypoplastic left heart syndrome

The postnatal survival of patients with congenital cardiac defects such as hypoplastic left heart syndrome (HLHS) is dependent on the patency of the ductus arteriosus and the presence of an unrestrictive atrial septal defect (ASD). We report a six week old infant with HLHS and tricuspid valve regurgitation with restrictive ASD. Transfemoral balloon atrial septostomy provided temporary relief but further attempts were not possible. A transhepatic venous approach to stent the atrial communication was technically successful. This approach may be the only access for certain infant cardiac catheterization interventions, permitting the use of a large delivery system (≥ 8 Fr).

Radiofrequency Catheter Ablation of Intractable Ventricular Tachycardia in an Infant Following Arterial Switch Operation

A full-term male neonate presented with cyanosis upon delivery and was subsequently diagnosed with d-transposition of the great arteries, ventricular septal defect, and restrictive atrial septal defect. Following initiation of intravenous prostaglandins and balloon atrial septostomy, an arterial switch operation was performed on day 3 of life. The postoperative course was complicated by intractable ventricular tachycardia that was refractory to lidocaine, amiodarone, esmolol, fosphenytoin, and mexiletine drug therapy. Ventricular tachycardia was suppressed with overdrive atrial pacing but recurred upon discontinuation. Seven weeks postoperatively, radiofrequency catheter ablation was performed due to hemodynamically compromising persistent ventricular tachycardia refractory to medical therapy. The ventricular tachycardia was localized to the inferior-lateral right ventricular outlet septum. The procedure was successful without complications or recurrence. Antiarrhythmics were discontinued after the ablation procedure. Seven days after the ablation, a different, slower fascicular rhythm was noted to compete with the infant's sinus rhythm. This was consistent with the preablation amiodarone having reached subtherapeutic levels given its very long half-life. The patient was restarted on oral beta blockers and amiodarone. The patient was subsequently discharged home in predominantly sinus rhythm with intermittent fascicular rhythm.