Subpulmonary Stenosis Research Articles

Left Ventricular Retraining and Double Switch in Patients With Congenitally Corrected Transposition of the Great Arteries

Congenitally corrected transposition of the great arteries (CC-TGA) is a complex form of congenital heart defect with numerous anatomic subgroups. The majority of patients with CC-TGA are excellent candidates for a double-switch procedure. However, in the absence of an unrestrictive ventricular septal defect or subpulmonary stenosis, the left ventricle (LV) may undergo involution and require retraining prior to double switch. The purpose of this study was to review our experience with patients having CC-TGA who required LV retraining prior to a double-switch procedure. This was a retrospective review of 24 patients with CC-TGA who were enrolled in an LV retraining program in preparation for a double-switch procedure. The median age at the time of enrollment for retraining was 11 months (range 1 month-24 years). The average left ventricle to right ventricle pressure ratio was 0.39 ± 0.07 prior to intervention. All 24 patients underwent placement of an initial pulmonary artery band (PAB) for LV retraining. Eighteen (75%) of the 24 patients underwent a double-switch procedure with no operative mortality. Of these 18 patients, 9 had a single PAB and 9 required a second band for retraining. Six patients have not undergone a double-switch procedure to date. Five patients are good candidates for a double switch and are 2 weeks, 3 weeks, 4 weeks, 8 months, and 35 months since their last PAB. One patient died from a noncardiac cause 26 months after PAB retightening. The 18 patients who underwent a double switch were followed for an average of 5 ± 1 years (range 0.1-10.3 years). There has been no late mortality, and only 2 patients required further reinterventions. The data demonstrate that LV retraining has been highly effective in this select group of patients with CC-TGA. The data also demonstrate that the results of the double-switch procedure have been excellent at midterm follow-up. These results suggest that LV retraining and double switch offer a reliable strategy option for patients with CC-TGA.

Dextrocardia and Situs inversus as part of Situs inversus totalis with Pulmonary and Sub-Pulmonary Stenosis, in a patient with Sinus Node Dysfunction

Dextrocardia and Situs inversus as part of Situs inversus totalis with Pulmonary and Sub-Pulmonary Stenosis, in a patient with Sinus Node Dysfunction

Senning operation as a palliative therapy for a girl with complex heart defect and Eisenmenger syndrome (RCD code: II-1A.4d)

Development of irreversible pulmonary hypertension in D-transposition of the great arteries (D-TGA) with ventricular septal defect (VSD) is a well-known phenomenon. Coexistence of left ventricular outflow tract obstruction (LVOTO) should theoretically protect the patient against pulmonary arterial hypertension (PAH). Application of the Blalock-Taussig shunt (B-TS) temporarily improves condition of the patient, but may contribute to irreversible PAH requiring palliative complex therapy. We present a case of a 18 year-old female with congenital complex heart defect: D-TGA with inflow VSD and subpulmonary stenosis, Eisenmenger syndrome, who was treated with several subsequent palliative cardiosurgical operations, including B-TS and Senning procedure and eventually, combined PAH-specific therapy, what resulted in stabilization of clinical status. JRCD 2016; 3 (1): 14–16

Interventricular membranous septal aneurysm: CT and MR manifestations.

Advanced cardiac imaging is a valuable method to investigate cardiac malformations. The detection of the interventricular membranous septum has clinical significance due to thrombogenic and arrythmogenic predisposition, as well as a role in obstructing the pulmonary flow. This review describes six clinical presentations in which advanced cardiac imaging has been the tool for evaluation, with special emphasis in CT angiography and cardiac MRI sequences.Teaching Points• The interventricular membranous septum can predispose patients to thrombogenic and arrythmogenic events.• Subpulmonic stenosis relates to the protrusion of the aneurysm into the right ventricle• During surgery, ventricular pressures of the opened heart become balanced, making the aneurysm less evident.

Anomalous Muscle Bundles Causing Subpulmonic Stenosis

Introduction: Muscular subpulmonic stenosis causing right ventricular outflow tract obstruction is an uncommon cardiac disease, especially when first diagnosed in adults. Diagnosis can be challenging when it is minimally symptomatic. Case Presentation: We report a 40-year-old man who was referred to cardiology clinic for work-up of dyspnea and a heart murmur found on his physical examination. A transesophageal echocardiogram (TEE) revealed a muscular subpulmonic stenosis and a small perimembranous ventricular septal defect (VSD) with left-to-right shunt and severe right ventricular outflow tract obstruction (RVOT). Right heart catheterization showed an estimated gradient of 75 mmHg across the two chambers due to subpulmonary stenosis. The pressure in the proximal right ventricular chamber was 100/25 mmHg and 25/17 mmHg in the distal chamber. Surgical correction was successfully performed. A postoperative TEE showed no residual gradients across the RVOT. Conclusions: Full echocardiographic examination of the right ventricle, including RVOT is essential for the diagnosis of this disease. Surgical correction appears to be a successful strategy for management of this rare condition in adults.

Subpulmonary Obstruction from Aneurysmal Ventricular Septum in a Child with Dextrocardia and Congenitally Corrected Transposition of the Great Arteries

A 3-year-old boy underwent evaluation for dextrocardia. Echocardiograms showed features of corrected transposition physiology, a perimembranous ventricular septal defect (VSD) (Fig. 1), and aneurysmal tissue beneath the pulmonary valve that caused severe subpulmonary stenosis (Figs. 2–4). Eighteen months after VSD closure and resection of the aneurysmal tissue, the patient was asymptomatic with only mild residual pulmonary outflow tract obstruction. Fig. 1. Two-dimensional echocardiograms A) in apical 4-chamber view from the right chest and B) in color-flow Doppler mode show a moderate-to-large ventricular septal defect (VSD). Fig. 2. Echocardiograms (subcostal 4-chamber views). A) In the morphologic left ventricle (MLV), aneurysmal tissue (arrow) protrudes into the pulmonary outflow tract. B) Color-flow Doppler mode reveals turbulent flow (arrow) in the pulmonary outflow tract. Fig. 3. A) Continuous-wave Doppler echocardiographic recording across the pulmonary outflow tract shows a peak velocity >5 m/s, suggesting severe obstruction. B) Angiogram (60° left anterior oblique view) from the morphologic left ventricle (MLV) ... Fig. 4. Cineangiographic image from the A) left lateral view reveals the morphologic left ventricle (MLV) and the pulmonary outflow tract obstruction from aneurysmal tissue (arrows). B) The 60° left anterior oblique view reveals a severely narrowed right ...

Congenital heart disease in the older adult: a scientific statement from the American Heart Association.

The population of adults with congenital heart disease (ACHD) has increased dramatically over the past few decades, with many people who are now middle-aged and some in the geriatric age range. This improved longevity is leading to increased use of the medical system for both routine and episodic care, and caregivers need to be prepared to diagnose, follow up, and treat the older adult with congenital heart disease (CHD). The predictable natural progression of CHD entities and sequelae of previous interventions must now be treated in the setting of late complications, acquired cardiac disease, multiorgan effects of lifelong processes, and the unrelenting process of aging. Despite the advances in this field, death rates in the population from 20 to >70 years of age may be twice to 7 times higher for the ACHD population than for their peers.1 This American Heart Association (AHA) scientific statement will focus on the older adult (>40 years old) with CHD. It is meant to be complementary to the 2008 American College of Cardiology (ACC)/AHA guidelines for ACHD and orient the reader to the natural history, ramifications of childhood repair, and late initial diagnosis of CHD in the older adult. This population with CHD is unique and distinct from both the pediatric and young adult populations with CHD. Much of the information we provide is from scientific research combined with clinical experience from longitudinal care. We emphasize that this is the beginning of a discussion regarding this rapidly growing population, and continued research aimed at the progression of disease and complications reviewed here is necessary to advance the field of ACHD with the scientific rigor it deserves. ACHD encompass a broad range of presentations. There are people who are diagnosed for the first time in adulthood, as well as those with prior palliative repair …

Reply to the letter from Finsterer and Stöllberger "Exhaustion or fatigability may not only be cardiac but also myopathic".

We are pleased to have received comments on our recently published manuscript ‘Unexpected finding in an adult with ventricular fibrillation and an accessory pathway: noncompaction cardiomyopathy’ [1]. Our patient is indeed not the first patient to be described with the combination of isolated left ventricular noncompaction cardiomyopathy (INVM) and Wolff–Parkinson–White syndrome; however, she is the first presenting an out-of-hospital cardiac arrest due to ventricular fibrillation. In the other reports, patients presented with either noncompaction cardiomyopathy or were diagnosed with this disorder by family screening. Feldt et al. [2] described a patient with a spongy trabecular pattern of the inner layer of both ventricles (though more pronounced in the right ventricle), but she also had dextrocardia, subpulmonary stenosis, and ventricular septum defect. In our introduction, we refer to the article of Engberding et al. [3] as the first to describe the presence of an enlarged left ventricle, thickening of the left ventricular wall, and huge sinusoids but a normal right ventricle. Hence, the only anomaly was an ‘isolated noncompaction of the left ventricle’. We agree that Feldt et al. were the first to describe a noncompaction cardiomyopathy (not isolated). A wide variety of ECG abnormalities and clinical findings can indeed be found in INVM patients, but there were only a few present in our case. It was the initial assumption on admission that ventricular fibrillation was induced by atrial fibrillation conducting rapidly across the accessory pathway. However, as we found another explanation and there was no documentation of atrial fibrillation during admission, we did not give oral anticoagulation. The patient is a hockey player without any restraints and it is therefore unlikely that she also had neuromuscular disorders. She was examined by a neurologist on admission, who did not find any abnormalities. We therefore think that further neurological evaluation is not indicated. So far, tachyarrhythmias have not been documented and genetic abnormalities have not been found.

Challenging Combination; Pseudocoarctation with Subaortic and Subpulmonic Stenosis

DOI: 10.4328/JCAM.995 Received: 09.04.2012 Accepted: 22.04.2012 Printed: 01.05.2015 J Clin Anal Med 2015;6(3): 366-8 Corresponding Author: Ahmet Kirbas, Medicana Camlica Hospital, Uskudar, Istanbul, Turkiye. F.: +9

Anatomy of the ventricular septal defect in outflow tract defects: Similarities and differences

ObjectiveThe study objective was to analyze the anatomy of the ventricular septal defect found in various phenotypes of outflow tract defects. MethodsWe reviewed 277 heart specimens with isolated outlet ventricular septal defect without subpulmonary stenosis (isolated outlet ventricular septal defect, 19); tetralogy of Fallot (71); tetralogy of Fallot with pulmonary atresia (51); common arterial trunk (54); double outlet right ventricle (65) with subaortic, doubly committed, or subpulmonary ventricular septal defect; and interrupted aortic arch type B (17). Special attention was paid to the rims of the ventricular septal defect viewed from the right ventricular side and the relationships between the tricuspid and aortic valves. ResultsThe ventricular septal defect was always located in the outlet of the right ventricle, between the 2 limbs of the septal band. There was a fibrous continuity between the tricuspid and aortic valves in 74% of specimens with isolated outlet ventricular septal defect, 66% of specimens with tetralogy of Fallot, 39% of specimens with tetralogy of Fallot with pulmonary atresia, 4.6% of specimens with double outlet right ventricle, 1.8% of specimens with common arterial trunk, and zero of specimens with interrupted aortic arch type B (P < .005). When present, this continuity always involved the anterior tricuspid leaflet. ConclusionsThe ventricular septal defect in outflow tract defects is always an outlet ventricular septal defect, cradled between the 2 limbs of the septal band. However, there are some differences regarding the posteroinferior and superior rims of the ventricular septal defect. These differences suggest an anatomic continuum from the isolated outlet ventricular septal defect to the interrupted aortic arch type B rather than distinct physiologic phenotypes, related to various degrees of abnormal rotation of the outflow tract during heart development: minimal in isolated outlet ventricular septal defect; incomplete in tetralogy of Fallot, tetralogy of Fallot with pulmonary atresia, and double outlet right ventricle; absent in common arterial trunk; and excessive in interrupted aortic arch type B.

Insights gained from three-dimensional imaging modalities for closure of ventricular septal defects.

Ventricular septal defects (VSDs) are the commonest group of congenital heart lesions, accounting for ≈30% of congenital heart defects in liveborn infants.1 Defects in the ventricular septum are classified according to their position when the ventricular septum is viewed from the right ventricle and these may vary in their location, size, and number.2,3 VSDs can occur as isolated lesions or as part of more complex disease, for example, transposition of the great arteries or double outlet right ventricle, where the location and size of such defects assume great importance in planning the optimal mode of surgical repair.4 In most cases, surgical closure remains the mainstay treatment for VSDs, but selected lesions may be amenable to catheter closure.5,6 Cross-sectional echocardiography is the most commonly used noninvasive diagnostic method to delineate the position of a VSD before surgery.7 Multiple sonographic cuts and cross-sectional sweeps of the region of interest are used to build a virtual reconstruction of the three-dimensional (3D) anatomy in the mind of the echocardiographer. However, for the surgeon who deals solely with 3D images there can remain an element of uncertainty when called to operate on the patient. The number, size, location of VSDs, and their relationship to adjacent structures are central for selecting whether the defects might be best closed via the tricuspid valve, via the semilunar valves, or via a ventriculotomy or whether these might not be readily approachable and a palliative pulmonary artery banding is desirable.8,9 In this review, we present key 3D echocardiographic projections and examples of different types of VSDs where the images have a direct effect on the approach taken to VSD closure. Comparison of 3D echocardiography (3DE) with other 3D imaging modalities is also described. Images are presented in an …

Streaming in Transposition of the Great Arteries by Using Cardiac Magnetic Resonance Imaging

A 6-month-old child with progressive cyanosis was referred to our service for assessment. Transthoracic echocardiography confirmed the diagnosis of transposition of the great arteries, a very large ventricular septal defect (VSD), and subpulmonary stenosis. The atrial septum was intact. The transcutaneous oxygen saturation levels measured 40% to 50% in room air with a hemoglobin value of 19 g/dL. Conventional treatment with an arterial switch and VSD closure was not possible, because it would result in neoaortic obstruction because of the significant subpulmonary stenosis. Additionally, the size of the VSD was such that achieving adequate septation of the ventricles remained questionable. To aid surgical planning, a cardiac magnetic resonance scan was performed to determine whether the low transcutaneous oxygen saturation levels were due to poor mixing of blood or limited pulmonary blood flow. The scan was performed on a commercial 1.5T scanner (Achieva; Philips Healthcare, Best, The Netherlands) with a 2-channel coil. The child was awake and self-ventilating with the use of a wrap-and-feed strategy. A validated highly undersampled 4D phase-contrast flow (4D flow) sequence1 was used to …

Transvenous cardiac resynchronization therapy in congenitally corrected transposition of the great arteries and progressive heart failure

Congenitally corrected transposition of the great arteries Transvenous cardiac resynchronization therapy Progressive heart failure A 24-year-old student with congenitally corrected transposition of the great arteries (ccTGA) who was unable to continue his study due to progressive signs of heart failure (NYHA III) was admitted to our department. He had a history of a double switch procedure including modified Senning- and arterial switch-operation as well as a surgical correction of the right pulmonary artery, Lecompte manoeuvre and resection of subpulmonary stenosis in the left ventricular outflow tract at the age of 6 years. Furthermore a ventricular septal defect was closed with a patch. During the surgical procedure the native systemic aortic valve was injured and replaced by a prosthetic aortic valve. Due to postsurgical third degree AV-block a DDD-pacemaker with epicardial leads was implanted in the abdomen. After exit-block of the epicardial right ventricular (RV) lead a transvenous RV-lead was implanted at the age of 16 years. At presentation NT pro-BNP levels were markedly elevated and 12lead-ECG showed permanent RV-stimulation and undersensing of the atrial lead (Panel A). Transthoracic echocardiography revealed a significantly dilatedand impairedsystemic ventricle with anejectionfraction of b25% and a normally functioning prosthetic aortic valve. Pacemaker interrogation exhibited malfunction (exit block and undersensing) of the atrial epicardial lead and increased thresholds of the transvenous RV-lead. Given the clinical presentation and the underlying ccTGA, the chronic dyssynchrony due to permanent RV-stimulation and atrio-ventricular dyssynchrony due to atrial undersensing were thought to be the most likely causes for the progression of heart failure. After a discussion with the patient and his family, a biventricular implantable cardioverter defibrillator (CRT-ICD) was implanted after complete removal of the abdominal pacemaker and partial removal of the transvenous RV-lead (Panel B; Film A): A defibrillator lead was placed in the apex of the systemic ventricle and an atrial lead was placed at the inferior free wall of the right atrium. Venography of the reconnected coronary sinus revealed a prominent infero-posterolateral vein (Panel C; Film B). Subsequently, a left ventricular pacing lead could be advanced successfully into this left ventricular vein and a CRT-ICD was implanted subpectorally (Panel D; Film C). Follow-up assessment at 6 months revealed a significant improvement in functional class (NYHA I; he was able to continue his studies), anincreaseofejectionfractionofthesystemicventricleandasignificant

Anesthesia and LEOPARD Syndrome: A Review of Forty-Nine Anesthetic Exposures

LEOPARD syndrome is a rare congenital disease that can manifest with cardiac anomalies, multiple lentigines, ocular hypertelorism, growth retardation, and deafness. The purpose of this case series was to review the most prominent comorbidities associated with LEOPARD syndrome, and describe perioperative outcomes in a series of patients undergoing anesthesia. Retrospective case series review Tertiary care institution Patients diagnosed with LEOPARD syndrome who underwent surgical procedures requiring anesthesia at this institution. The medical and anesthesia records of patients with LEOPARD syndrome were reviewed. Demographic information, clinical features of LEOPARD syndrome, comorbidities, intraoperative and postoperative events and complications were recorded. A systematic literature review also was conducted. Nine patients with LEOPARD syndrome underwent 49 procedures under general anesthesia (n = 40) or monitored anesthesia care (n = 9). The majority of operations were related to correction of cardiac anomalies (n = 20). The most common cardiac malformations were ventricular septal hypertrophy and pulmonary (or subpulmonary) stenosis, and major perioperative complications were related to severe arrhythmias and/or cardiac decompensation. Dominant pathology associated with perioperative complications in patients with LEOPARD syndrome is related to cardiac disease. A large proportion of patients with this condition have ventricular septal hypertrophy, which tends to progress with age; therefore, these patients undergoing anesthesia should have recent cardiologist evaluation.

Tetralogy of Fallot: nosological, morphological, and morphogenetic considerations

It is timely, in the 125th anniversary of the initial description by Fallot of the hearts most frequently seen in patients presenting with "la maladie bleu", that we revisit his descriptions, and discuss his findings in the light of ongoing controversies. Fallot described three hearts in his initial publication, and pointed to the same tetralogy of morphological features that we recognise today, namely, an interventricular communication, biventricular connection of the aorta, subpulmonary stenosis, and right ventricular hypertrophy. In one of the hearts, he noted that the aorta arose exclusively from the right ventricle. In other words, one of his initial cases exhibited double-outlet right ventricle. When we now compare findings in hearts with the features of the tetralogy, we can observe significant variations in the nature of the borders of the plane of deficient ventricular septation when viewed from the aspect of the right ventricle. We also find that this plane, usually described as the ventricular septal defect, is not the same as the geometric plane separating the cavities of the right and left ventricles. This means that the latter plane, the interventricular communication, is not necessarily the same as the ventricular septal defect. We are now able to provide further insights into these features by examining hearts prepared from developing mice. Additional molecular investigations will be required, however, to uncover the mechanisms responsible for producing the morphological changes underscoring tetralogy of Fallot.

The essential echocardiographic features of tetralogy of Fallot

This presentation will demonstrate the essential features of tetralogy of Fallot in the infant and child before surgery, as well as some noteworthy features in the foetus. The four features, namely, subpulmonary stenosis, ventricular septal defect, aortic override, and right ventricular hypertrophy, can all be easily demonstrated by echocardiography. In addition, morphology of the pulmonary valve and the main and branch pulmonary arteries can be seen. The position of the coronary arteries and the major variants of proximal coronary anatomy can be defined. The arch anatomy and the presence of associated major aortopulmonary collateral arteries can be defined. All these features can be demonstrated in the foetus as well, after the first trimester, and the presence of major aortopulmonary collateral arteries can be seen more clearly because the lungs, being fluid filled, aid in ultrasound and do not provide the barrier that the air-filled lung presents after birth.

A 72-year-old woman with an uncorrected tetralogy of Fallot presenting with possible pulmonary endocarditis: a case report.

IntroductionTetralogy of Fallot is one of the most common forms of cyanotic congenital heart disease and in the absence of surgical correction it has an elevated early mortality, with most patients dying in childhood.The authors reported this case because of the unusual course of an uncorrected tetralogy of Fallot. There are only a few reports of patients with an uncorrected tetralogy of Fallot who reach an advanced age and to the best of our knowledge this is the first case report of a possible endocarditis in a patient with an uncorrected tetralogy of Fallot who is older than 70 years.Case presentationThe authors present a case of a 72-year-old Caucasian woman with uncorrected tetralogy of Fallot who was admitted with fever and heart failure to our Cardiology Department with possible infective endocarditis.ConclusionsThe longevity of this patient is probably due to the association between a large ventricular septal defect, a balanced subpulmonary stenosis and to the presence of systemic hypertension. After empiric antibiotic therapy, the patient was discharged and no surgical intervention was performed due to her previous benign evolution.

Palliative Arterial Switch as First-line Treatment Before the Fontan Procedure in Patients With Single-ventricle Physiology and Subaortic Stenosis

Introduction and objectivesThere are several techniques for the palliative treatment of patients with single-ventricle physiology, ventriculoarterial discordance and subaortic stenosis. The Fontan procedure relies on optimal initial palliation to avoid the development of subaortic stenosis (as well as ventricular hypertrophy and diastolic dysfunction). MethodsWe present seven patients with single-ventricle physiology, transposition of the great arteries and subaortic stenosis, with low systemic output and high pulmonary flow, aged 21 to 383 days (median, 75) and weighing between 3.4 and 9.6kg (median, 4.2). All were treated with a palliative arterial switch, thus “switching” their subaortic stenosis to subpulmonary stenosis. Six patients also underwent aortic arch surgery, 4 an atrial septectomy, and 1 a subaortic membrane resection. ResultsOne patient died after surgery, another developed recoarctation, which was treated with an angioplasty, 3 patients reached the Glenn stage and 2 the Fontan stage. All had good ventricular function. ConclusionsA palliative switch is an effective initial treatment for single-ventricle physiology with transposition of the great arteries and subaortic stenosis. This complex initial technique produces good results and allows the patient to progress to the Glenn or Fontan stage.

Switch arterial paliativo como primer tiempo hacia Fontan en pacientes con fisiología univentricular y estenosis subaórtica

Introduction and objectivesThere are several techniques for the palliative treatment of patients with single-ventricle physiology, ventriculoarterial discordance and subaortic stenosis. The Fontan procedure relies on optimal initial palliation to avoid the development of subaortic stenosis (as well as ventricular hypertrophy and diastolic dysfunction). MethodsWe present seven patients with single-ventricle physiology, transposition of the great arteries and subaortic stenosis, with low systemic output and high pulmonary flow, aged 21 to 383 days (median, 75) and weighing between 3.4 and 9.6kg (median, 4.2). All were treated with a palliative arterial switch, thus «switching» their subaortic stenosis to subpulmonary stenosis. Six patients also underwent aortic arch surgery, 4 an atrial septectomy, and 1 a subaortic membrane resection. ResultsOne patient died after surgery, another developed recoarctation, which was treated with an angioplasty, 3 patients reached the Glenn stage and 2 the Fontan stage. All had good ventricular function. ConclusionsA palliative switch is an effective initial treatment for single-ventricle physiology with transposition of the great arteries and subaortic stenosis. This complex initial technique produces good results and allows the patient to progress to the Glenn or Fontan stage.Full English text available from:www.revespcardiol.org/en

Subpulmonary Stenosis Assessed in Midtrimester Fetuses With Tetralogy of Fallot: A Novel Method for Predicting Postnatal Clinical Outcome

This study aimed to determine whether quantification of subpulmonary stenosis (SPS) in tetralogy of Fallot (TOF) in the second-trimester fetus can predict postnatal clinical outcome measured by pulmonary valve size and/or timing or type of intervention. The study retrospectively identified fetuses with TOF from 1998 to 2010 diagnosed at 26 weeks gestation or earlier. The data evaluated included pre- and postnatal pulmonary valve z-scores (PVZ). To quantify fetal SPS, the authors created a novel index, the SPS/DAO ratio, a ratio of the minimum infundibular diameter to the descending aorta diameter (DAO). Multiple linear regression was used to predict postnatal PVZ from prenatally determined parameters, including SPS/DAO. Fetal parameters were analyzed by logistic regression for association with postnatal outcomes, namely, timing of surgery (<1 month), used as a surrogate for severity, and type of surgery [transannular patch (TAP) vs valve sparing surgery]. A total of 23 fetuses met the inclusion criteria. The mean gestational age was 21.8 ± 1.9 weeks (range, 16.6-25.4 weeks). There was excellent correlation between predicted and measured PVZ (r = 0.82; p < 0.0001) using the following derived equation: -3.68 + (0.91 × prenatal PVZ) - (4.44 × SPS/DAO) - 3.19 (prenatal PVZ × SPS/DAO). An SPS/DAO value lower than 0.5 had 100 % sensitivity and 56 % specificity for repair before the age of 1 month, and a value lower than 0.47 had 100 % sensitivity and 75 % specificity for TAP repair. Prenatal PVZ and the SPS/DAO ratio at 26 weeks gestation or earlier can reliably predict postnatal PVZ in fetuses with TOF. Quantification of SPS with the SPS/DAO ratio identifies patients who may require early intervention secondary to disease severity and may predict the type of repair, thereby influencing prenatal counseling.