|
 
 |
| FOCUS ISSUE - CONGENITAL HEART DISEASE |
|
| Year : 2020 | Volume
: 4
| Issue : 3 | Page : 276-286 |
|
Echocardiography to Evaluate Pulmonary Stenosis
Smita Mishra1, Praneet Lele2
1 Department of Pediatric Cardiology, Manipal Hospital Dwarka, Delhi, India
2 Consultant Pediatric Cardiologist, Kingsway Hospital, Nagpur, Maharashtra, India
| Date of Submission | 16-Aug-2020 |
| Date of Acceptance | 18-Oct-2020 |
| Date of Web Publication | 18-Dec-2020 |
Correspondence Address:
Dr. Smita Mishra
190, First Floor, Sukhdev Vihar, New Delhi - 110 025
India
 Source of Support: None, Conflict of Interest: None
DOI: 10.4103/jiae.jiae_47_20
Congenital pulmonary stenosis (PS) is a common term for lesions causing right ventricular outflow obstruction. It can be further classified as the valvar, supravalvar, and infundibular PS. The PS may often present with other congenital heart diseases. In this article, echo imaging of isolated PS has been discussed. It is imperative to know that the guideline for intervention in isolated PS is totally based on echocardiography. Echocardiographic guidance is required for the selection of procedure, hardwares, evaluation of the outcome of the procedure, and long-term prognosis. Keywords: Infundibular pulmonary stenosis, supra valvar pulmonary stenosis, valvar pulmonary stenosis
How to cite this article:
Mishra S, Lele P. Echocardiography to Evaluate Pulmonary Stenosis. J Indian Acad Echocardiogr Cardiovasc Imaging 2020;4:276-86 |
How to cite this URL:
Mishra S, Lele P. Echocardiography to Evaluate Pulmonary Stenosis. J Indian Acad Echocardiogr Cardiovasc Imaging [serial online] 2020 [cited 2023 Mar 28];4:276-86. Available from: https://jiaecho.org/text.asp?2020/4/3/276/303940 |
| Introduction | |  |
Congenital pulmonary stenosis (PS) refers to lesions accountable for congenital right ventricular outflow obstruction (RVOTO).[1],[2] Acquired PS is rare and often caused by compression by external or internal structures. RVOTO is classified as valvular PS (VPS), subvalvular (infundibular pulmonary stenosis [IPS]), supravalvular PS (SVPS), and peripheral PS (PPS), according to the location of the obstruction. In this article, we are going to discuss isolated PS, its anatomical considerations, applied echocardiography in decision-making in terms of intervention and follow-up.[1],[2],[3],[4] Almost all guidelines for the management of PS are based on echocardiography [Table 1]. The prenatal echocardiographic scoring system has been proposed to prognosticate the postnatal outcome of fetuses with critical PS with the intact ventricular septum (CrPS/IVS) [Table 2].[5] | Table 2: Fetal echocardiographic criteria in 2nd trimester to assess the outcome of critical pulmonary stenosis or pulmonary atresia intact ventricular septum
Click here to view |
Incidence
The incidence of PS has been reported as 0.6–0.8/1000 live births, 5.8% of all congenital heart defects (CHDs), and almost 50% of the patient population with CHDs may also have PS.[1],[2],[6],[7]
Congenital dysmorphic syndromes and pulmonary stenosis
[Table 3] is a brief summary of congenital dysmorphic syndromes associated with the PS. PS associated with congenital syndromes is usually complicated by the presence of the dysplastic pulmonary valve (PV), multiple sites of RVOTO (subvalvular membrane, SVPS, and PPS) and may not be amenable for balloon pulmonary valvotomy (BPV).[1],[5],[8] Noonan's Syndrome is an autosomal dominant genetic condition, known to associate with varying level of right ventricular outflow obstruction in about 50% of its patient population. Owing to the presence of hypoplastic pulmonary annulus and dysplastic PV cusps, they often respond poorly to cath interventions. PS may also be associated with atrial septal defect (10%), asymmetrical septal hypertrophy (10%), ventricular septal defect (VSD) (5%), persistent ductus arteriosus (3%), peripheral pulmonary stenosis and mitral valve prolapse.[4],[9]
Clinical diagnosis
Clinical features of PS are narrated in [Table 4].[1],[2],[5],[7]
| Adaptive Changes of Right Ventricle in Response to the Pulmonary Stenosis | | |
In the natural history of unresolved moderate to severe PS, echocardiography provides us evidence of the interplay of several adaptive and maladaptive changes. The presence of concentric hypertrophy coupled with predominantly end-systolic and early-diastolic flattening of the IVS in M-Mode echocardiogram, suggest adaptive changes in response to longstanding right ventricular (RV) pressure overload.[10] The tricuspid valve (TV) inflow velocities may show a restrictive pattern, but systolic parameters remain in the normal range. After a threshold when contractility can no longer overcome heightened afterload, maladaptive remodeling (heteromeric adaptation) takes-over. The combined onset of eccentric hypertrophy, progressive RV dilatation, and desynchrony observed in echo-imaging reflect catastrophic RV functional deterioration, eventually culminating into clinical deterioration.[1],[2],[10],[11]
Ventricular interdependence and left ventricular (LV) response - Hypertensive RV leads to IVS shifting, altered LV geography, lower values for the cardiac index, indexed LV end-diastolic volume, ejection fraction (EF) etc., and LV hypertrophy.[1],[12]
| Valvular Pulmonary Stenosis | | |
Morphological consideration and echocardiography
RV is a tripartite (inlet, cavity, outlet portion) anterior-most chamber of the heart. Between the cavity and PV, a muscular sleeve (conus or infundibulum) exists, instigating fibrous discontinuity between PV and TV [Figure 1]a,[Figure 1]b,[Figure 1]c.[1] The main pulmonary artery (MPA) connects the ventricle to branch pulmonary arteries (right and left branch PA) and has a bicornuate appearance due to distal bifurcation [Figure 1]c. Essentially, RV muscle mass (RV to LV free wall ratio − 1:3) and systolic pressure (1/4th–1/5th of LV systolic pressure) are considerably lower than LV. PA systolic pressure is almost equal (within 10 mmHg) to RV systolic pressure; diastolic pressure of PA is very low due to less muscularity and high elasticity of PV, and it falls further in the presence of pulmonary regurgitation (PR).[1],[13],[14],[15] In severe PS, RV responds with muscular hypertrophy, disproportionately rise in RV systolic pressure in comparison to PA creating a significant gradient. The severity of PS is decided by this gradient. | Figure 1: Pulmonary valve (morphological points): Parasternal short axis view – (a) Basal muscular ring (1); body of cusps in between the basal ring and Sino-tubular junction (2); sinotubular junction or cranial attachment (3) a site of supravalvular PS. Red stars are points for PV annular measurement; TV attachment and muscular sleeve between TV and PV (yellow star and yellow broken line). (b) Three-dimensional CT angio showing pulmonary valve from outside (pink lines demarketing extent of pulmonary valve). (c) Morphological points - A parasternal short axis view. Classical “Cup and saucer” view due to obliquity of infundibulum a muscular sleeve distancing TV and PV; shared wall of left ventricular outflow with RVOT (green), MPA (yellow), RPA (violet); various sites of possible obstruction are shown (infundibular, valvular, supravalvular, bifurcation and branch pulmonary arteries.). PS: Pulmonary stenosis, PV: Pulmonary valve, TV: Tricuspid valve, CT: Computed tomography, RVOT: Right ventricular outflow obstruction, MPA: Main pulmonary artery, RPA: Right pulmonary artery
Click here to view |
The goals of echo-imaging and views required to evaluate PS are described in [Table 5] and [Table 6], respectively.
In most of the autopsy series of VPS, PV were reported to be trileaflet.[1],[13] However, Gikonyo et al. found unicommissural (16%), were bicuspid (10%), tricuspid (6%), valve with hypoplastic annulus (6%) and dysplastic cusps (19%) and in rest (42%) they could not define morphology.[14]
- Pulmonary cusp: Isolated VPS usually presents with thickened and domed cusps due to the commissural fusion, tethering of PV at sinotubular junction [Figure 2]. Infrequently, the PV may have dysplastic cusp and annular hypoplasia or an obstructive membrane at the supra or subpulmonary area
In echo, it is difficult to observe the enface view of PV and the number of cusps or PV area.[1],[15] Post stenotic dilatation of MPA, a hallmark of valvar PS is seen irrespective of the severity of PS [Figure 3]a and [Figure 3]b.[16] Absence of it or mild narrowing of MPA instead, suggests the possibility of covert SVPS [Figure 3]c and [Figure 3]d. The measurement of PV annulus (inner wall to inner wall distance) must be taken at the hinge-point plane, and Z score must be obtained [Figure 1]a. Z scores for the cardiac dimensions are available online.
- Z score of PV gives an idea about: (1) capacity of native PV to handle RV output; (2) it helps in the balloon sizing in preparation of BPV. PV annulus is mostly muscular and can be stretched up to 120%–140%. However, severe PR may cause RV dilatation and desynchrony in the long run. Therefore, it is better to avoid balloon-annulus ratio above 1.2:1. It may not achieve a better PV area and may worsen the PR.
- Z Score values of TV annulus (TVA):[4],[14],[15] RV cavity volume and EF are important determinants of outcome of the intervention, which cannot be measured by Simpson's method due to the triangular shape cavity of RV.[1] Literature suggests, TV Z score corresponds well with RV volume.[1],[17],[18],[19] The TVA is a dynamic structure and can be measured in diastole by demarcating hinge point to hinge point distance carefully, in a apical four chamber view [Figure 4]. In severe neonatal PS, RV can be musclebound tripartite or may lack cavity (bipartite). Early removal of RVOTO helps in reverting the unfavorable effects of pressure overload on RV [Figure 5]a and [Figure 5]b. A grossly dysplastic TV may have Ebstenoid or Ebstein's anomaly of TV leaflets as well as anomalous papillary muscles of TV.[1],[20] In our experience, CrPS with severely dysplastic TV may present with massive cardiomegaly in a cyanosed newborn, requiring urgent fixing of TV in addition to the pulmonary valvotomy
Poor transthoracic window in older patients leads to poor imaging, and trans-oesophageal echo can be used for better understanding [Figure 6]a,[Figure 6]b,[Figure 6]c.
- Post procedurefunctional PS: Hypertrophied septal and parietal bands (infundibular PS-IPS), if not noticed pre-procedure, may cause suprasystemic RV pressure and low cardiac output (suicidal RV) after a BPV procedure.[21]
- Fetal presentation of severe PS (CrPS with the intact ventricular system - CrPS/IVS): Fetal critical PS/IVS or pulmonary atresia/IVS (PA/IVS), may cause a variable degree of hypoplasia of the TV, PV, and RV resulting into postnatal duct dependence [Figure 7]a,[Figure 7]b,[Figure 7]c,[Figure 7]d,[Figure 7]e. Early intervention in fetal or postnatal life, gives an impetus to borderline RV (TVA Z score <−2) to have better ejection volume and growth; eventually, few of these neonates may escape the univentricular palliation.[4],[5],[11],[19] Ballooning of PV may not always improve the systemic saturation, requiring continuous prostaglandin infusion or additional procedures like duct stenting or systemic to pulmonary shunt surgery. To some extent, suboptimal results can be predicted by Z score of TVA.[22] Echocardiographic criteria has been described to make prenatal prediction of the eventual outcome of Cr-PS/IVS or PA/IVS [Table 3].[4]
- Differential diagnosis of CrPS:
- Membranous pulmonary atresia/IVS (no antegrade flow at PV)
- Ebstein's anomaly of TV with functional pulmonary atresia (Severe tricuspid regurgitation (TR) leads to non-opening of PV as RV fails to overcome neonatal pulmonary arterial hypertension [PAH]).
- VSD, PS where VSD is not seen due to dynamic closure of VSD by TV tissue [Figure 5]c.
 | Figure 2: Subcostal view – (probe tilted up from classical 5C subcostal coronal view) tripartite right ventricle (inlet; trabecular cavity; outlet portion) valvular pulmonary stenosis with secondary hypertrophy of trabecula septo marginalis - trabecula septo-marginalis (y) is hypertrophied. PV: Pulmonary valve, SB: Septal band, RA: Right atrium, TV: Tricuspid valve
Click here to view |
 | Figure 3: Comparision of supravalvular region (parasternal short axis view): (a) 7-month-old infant with pure valvar pulmonary stenosis and post stenotic dilatation of main pulmonary artery. (b) across the PV 86 mmHg; (post-BPV 2 years follow up gradient - 15 mmHg). (c) 9 months old infant with valvar pulmonary stenosis and supravalvar tethering and narrowing. Post-BPV gradient 35 mmHg; (d) gradient across pulmonary valve = 85 mmHg after 2 years. PV: Pulmonary valve, BPV: Balloon pulmonary valvotomy, RVOT: Right ventricular outflow tract, RA: Right atrium, LA: Left atrium, Ao: Aorta, RPA: Right pulmonary artery, LPA: Left pulmonary artery, TV: Tricuspid valve, AV: Aortic valve
Click here to view |
 | Figure 4: Measurement of tricuspid valve annulus (red stars-show hinze point in diastole) and right atrial dimensions. RV: Right ventricle, RA: Right atrium, LA: Left atrium, LV: Left ventricle, MV: Mitral valve
Click here to view |
 | Figure 5: Apical 4 chamber view. (a) 1-year-old baby with PS. He underwent balloon pulmonary valvuloplasy for CrPS at 7th day of life (SPO2 = 67%). Restrictive VSD was missed then due to dynamic complete closure of VSD by prolapsing TV. Aneurysmal atrial septum (grey arrow), shifted towards left atrium (grey arrow). RV cavity muscle bound (yellow arrow)-TV measurement (hinge points marked with grey stars) Z-score was minus 2.1, tripartite RV, PV Z score minus 2. (b) 1-year post BPV SPO2 was 92%, TV Z score minus 1.2; (c) Parasternal short axis view from same child. Complete closure of dynamic TV accessory tissue, prominent septal and parietal band and hypoplastic PV annulus and MPA; (d) Classical spectral Doppler showing high velocity signal and hyper echoic dagger shape signal suggestive of dynamic subvalvar component. (Gradient across PV increased due to dysplastic PV and subvalvular obstruction; he underwent biventricular RVOT patch repair at the age of 2 years). PS: Pulmonary stenosis, CrPS: Critical pulmonary stenosis, VSD: Ventricular septal defect, TV: Tricuspid valve, RV: Right ventricular, PV: Pulmonary valve, BPV: Balloon pulmonary valvotomy, MPA: Main pulmonary artery, RVOT: Right ventricular outflow obstruction
Click here to view |
 | Figure 6: Trans esophageal echo mid esophageal (0°) View: (a) Normal pulmonary valve; (b) trans gastric view showing pulmonary valve, mild hypertrophy of sub valvular muscle bundle (white arrow). (c) Severe pulmonary stenosis, hypertrophied subvalvar muscle bundles. AV: Aortic valve, RVOT: Right ventricular outflow tract, MPA: Main pulmonary artery, RPA: Right pulmonary artery, LPA: Left pulmonary artery, PA: Pulmonary artery, Ao:Aorta, PV: Pulmonary valve
Click here to view |
 | Figure 7: Critical pulmonary stenosis in at 32 weeks of gestation: (a) noncontractile right ventricle (left ventricular systolic contraction), nonrestrictive patent foramen ovale (PFO) (yellow arrow); (b) hyperechoic papillary muscle of TV; (c) TR; (d) modified parasternal short axis view: color Doppler: turbulent jet across the PV; (e) High velocity signal across the PV: 288 cm/s (gradient 33 mmHg). TV: Tricuspid valve, TR: Tricuspid regurgitation, PV: Pulmonary valve, RV: Right ventricle, RA: Right atrium, LA: Left atrium, LV: Left ventricle
Click here to view |
Primary pulmonary hypertension also presents with systemic/supra systemic RV pressure with eccentric RVH. Clinically, there is no ejection murmur, but a high pitched early diastolic murmur of PR will be heard and usually no cyanosis.[23]
| Severity of Pulmonary Stenosis | | |
It is defined based on the Doppler peak gradient as follows:[24],[25]
- Severe PS - A gradient of 64 mmHg and above or if RV systolic pressure has been estimated as ≥2/3rd of systemic, systemic, or suprasystemic
- Moderate PS: PS gradient >36–<64 mmHg is considered as moderate
- Mild PS: PS is considered mild if the gradient is below 36 mmHg.
| Fallacies of Doppler Echo-imaging | | |
Accurate recording and interpretation of Doppler gradient are essential in decision making. Following fallacies are often seen during the echo-evaluation:
- Underestimation of the severity of PS:
- Low gain-setting
- Poor alignment of spectral Doppler cursor (inappropriate Doppler angle) or poor echo window
- Multiple level of stenosis; (TR jet gradient may correlate better)
- In PS/PAH physiology like the presence of a large shunt beyond the PV (aortopulmonary window or patent ductus arteriosus) or transient pulmonary hypertension of neonate
- RV dysfunction.
- Overestimation of the severity of PS:
- High gain setting of spectral Doppler or not choosing well-enveloped signals (ghosting artifact)
- Systolic high-velocity jet of restrictive doubly committed VSD getting mixed with PS gradient
- Systolic high-velocity jet of perimembranous VSD may cause overestimation of infundibular PS.
Therefore, it is important to have a good 2D and color Doppler echo-image before recording the gradient. The anatomical site and extent of obstruction on 2D as well as restriction of the turbulent jet (color Doppler) must be noticed. Spectral Doppler cursor must be aligned to the turbulent jet to minimize the Doppler angle [Table 6].
Evaluation of the systolic and diastolic function of the right ventricle in pulmonary stenosis
[Table 7] summarizes echocardiographic evaluation of RV function [Figure 8] and [Figure 9].[26],[27],[28],[29],[30],[31],[32] | Figure 8: Right ventricular systolic functional evaluation: (a) Fractional area shortening and right atrial size. (b) Tricuspid annular plane systolic excursion. (c) Myocardial performance index; (d) Tissue Doppler recording - Tei Index: Normal value: 0.32–0.24. RV: Right ventricle, RA: Right atrium, LA: Left atrium, LV: Left ventricle, TAPSE: Tricuspid annular plane systolic excursion, IVCT: Iso volumic contraction time, IVRT: Isovolumic relaxation time, RVET: Right ventricular ejection time, MPI: Myocardial performance index
Click here to view |
 | Figure 9: Evaluation of right atrial pressure: (a) Right to left shunt across the patent foramen ovale (arrow); (b) Noncollapsing inferior vena cava suggestive of right atrial pressure >8 mmHg (red arrow); (c) M-mode Echo suggests increased right ventricular free wall thickness (red arrow) and flattening of septum (green arrow)
Click here to view |
 | Table 7: Functional abnormality of right ventricle in pulmonary stenosis [Figure 8]
Click here to view |
| Medical Follow up and Outcome | | |
Follow up echo-studies are done to see gradient, RV/LV dysfunction, development of the complications like TR, infective endocarditis, thrombosis, subaortic stenosis. etc.
- Fetal PS: For mild PS (normal pulmonary and tricuspid annulus), no specific protocol needed; For moderate to severe PS (± hypoplasia of TV, PV, and RV) close observation is recommended to detect cardiac dysfunction and pericardial effusion [Table 3]
- Postnatal PS: Children with mild PS may need to follow-up every 1–2 years. While patients with moderate PS may need more frequent follow-up. Those with Severe PS must go for intervention. Lange et al. observed that more than 80% of patients did not need intervention if PS was mild (pulmonary gradient <25 mmHg [5% of cohort] <40 mmHg [20% of cohort]) to begin with.[29] If initial gradient was between 40 and 49 mmHg, average rise in gradient was up to 8.6 mmHg/year.[29] Lueker et al., reported a decrease in PS gradient in a cohort presented with mild PS[33]
- Postprocedure assessment:[1],[34],[35] RV systolic pressure usually goes significantly down after the BPV. A transient but severe rise of RV pressure is seen in the presence of infundibular muscular hypertrophy and can be treated medically. Surgical intervention rarely needed. Transient RV systolic dysfunction may also be seen particularly in the presence of severe PR or TR. RV diastolic dysfunction may persist transiently (children) or for an extended period in grownup patients. PR may be high, as discussed above and may not be tolerated [Figure 10]. The babies who had pre-procedure TV Z score <−2 or PV Z score <−4 may have suboptimal outcomes after BPV and must be evaluated further for the requirement of additional procedure.
 | Figure 10: Post ballooning procedure residual lesions: (a) Severe dilatation of right atrium and right ventricle (30 years post balloon pulmonary valvotomy). (b) Reverse flow in main and branch pulmonary arteries s/o severe pulmonary regurgitation. RV: Right ventricle, RA: Right atrium, LA: Left atrium, LV: Left ventricle
Click here to view |
Echocardiographic criteria for severe pulmonary regurgitation
- The width of the vena contracta is >50% of the width of the PV annulus
- Diastolic flow reversal in branch PA
- The duration of PR exceeds 2/3rd of the duration of diastole
- Pressure half time <100 ms.[36] We have seen one adult patient who presented with severe RV dysfunction and atrial fibrillation after 30 years of BPV [Figure 10]a and [Figure 10]b.
d. Patient with PS (pre- or postintervention) presenting with fever: A careful observation would be needed to rule out pericardial effusion and any vegetation in a sick symptomatic baby.
| Exercise Testing Coupled with Transthoracic Echo | | |
Exercise testing coupled with transthoracic echo may help in decision making for intervention or for sports advice for the adolescent and adults who become symptomatic on exertion despite of moderate gradient at RVOT. The post-intervention capacity to exercise improves in children but not in adults.[37],[38]
| Infundibular Pulmonary Stenosis | | |
(Also see [Table 1] for guidelines on timing and mode of intervention; and [Table 6] for echocardiographic views.).
IPS is often a form of progressive subvalvular obstruction of RVOT. It can be classified as high and low IPS. It is associated with perimembranous VSD in 80%–90% patients. Gasul's hypertrophy of outlet septum in the presence of large perimembranous VSD, converts a shunt physiology into the acquired tetralogy of Fallot.[39] Partial closure of VSD and progressive conal septum hypertrophy, presents as IPS in adulthood. Associated abnormalities like subaortic membrane may also be found or may develop later in the natural history. One of our patients was operated for IPS at the age of 2 years and presented with syncope and underlying severe subaortic stenosis at the age of 10 years.
Assessment of the severity of infundibular pulmonary stenosis
Like VPS, recording of velocity and gradient by spectral Doppler is pivotal. Fallacies discussed VPS, are applicable for IPS also. To minimise Doppler angle, selection of view is utmost important [Table 4]. Multiple views must be used to record the gradient.
| High Infundibular Pulmonary Stenosis | | |
It is also known as “napkin-ring stenosis” or “high double chamber RV” and “stenosis of infundibular ostium” [Figure 11]a and [Figure 11]b.[40] | Figure 11: Two-dimensional parasternal short axis view, infundibular pulmonary stenosis, hypertrophied parietal and septal bands (red arrow); in colour Doppler (red arrow), see red colour suggestive of direction of flow towards the probe. AA: Ascending aorta
Click here to view |
High IPS is caused by hypertrophied septal and parietal bands at ventriculo-infundibular (conal) junctions. The muscular ridge formed by hypertrophied muscle bundles creates two chambers (upper low pressure and lower high-pressure chamber) within the confines of the right ventricle. The upper chamber has also been designated as “moghul” chamber. Anatomical evaluation of RV cavity, moderator band (MB), and infundibulum are essential.
| Double Chamber Right Ventricle (Low Infundibular Stenosis) | | |
Double chamber right ventricle is also considered as the septation of RV [Figure 12]a,[Figure 12]b,[Figure 12]c,[Figure 12]d. The septation happens due to the presence of an anomalous muscular bundle, which starts from the apex of RV, parallel to IVS and gets attached to the free wall of RV. These bundles are distinct from the trabecula septo-marginalis (TSM) and the MB; they vary grossly in its location and morphology.[41],[42] | Figure 12: Low infundibular PS: (a) Modified parasternal long axis view: Diastole-low infundibular stenosis (grey arrow, flat IVS Green arrow); (b) Systole-spectral Doppler across the stenotic area, gradient: 119 mmHg – Unlike the usual PS, Doppler signal of low infundibular PS are seen above the baseline, indistinguishable from VSD signals. Anatomical narrowing gives the clue. (c) Modified PLAX view (same patient): Systole - turbulent jet can be seen (red arrow); See IVS movement towards LV below the stenosis (yellow arrow); (d) turbulent jet in Colour Doppler. PS: Pulmonary stenosis, PLAX: Parasternal long axis, VSD: Ventricular septal defect, IVS: Intact ventricular septum. AV: Aortic valve, LA: Left atrium, LV: Left ventricle
Click here to view |
| Supra Valvar Pulmonary Stenosis | | |
Congenital SVPS is rarer than post-surgery SVPS. A meta- analysis of 34 articles, only 20 (6%) patients had congenital SVPS. About 3.6% of patients of this group were syndromic, while all other patients had acquired SVPS [Table 2].[43]
Guidelines for intervention: [Table 1] and echocardiographic views [Table 4] have been described earlier in the article.
Anatomical type of supravalvular pulmonary stenosis
- Single stenosis of either of MPA, right pulmonary artery (RPA), left pulmonary artery (LPA) no other branch involvement [Figure 13]a,[Figure 13]b,[Figure 13]c and [Figure 14]a,[Figure 14]b,[Figure 14]c
- Stenosis at confluence extending up to the origin of branch PA, rest of RPA/LPA remain normal
- Multiple stenosis of distal branches and no involvement of central PA
- A combination of central and peripheral involvement is seen.[43],[44]
 | Figure 13: Parasternal short axis view: Supra valvular pulmonary stenosis in a 5-year-old child; (a) two-dimensional echo showing supravalvar pulmonary stenosis. (b) On color flow mapping, turbulent jetacross the pulmonary valve seen; (c) continuous wave Doppler: Accelerated velocity across the supra valvar pulmonary stenosis (gradient = 56 mmHg)
Click here to view |
 | Figure 14: Accidental detection of left axillary murmur in 4-year-old female patient. (a) Two-dimensional echo - parasternal short axis view) left pulmonary artery stenosis at origin; (b) three dimensional computed tomography pulmonary angiogram showing discrete narrowing of left pulmonary artery. (c) Continuous flow (diastolic spill - black arrow) across the stenotic area
Click here to view |
| Peripheral Pulmonary Stenosis | | |
Discrete stenosis of one of the branch PA may be isolated diseases and need to be evaluated for evidence of diversion of blood (dilatation of contralateral artery), pressure overloadand function of RV [Figure 15]a and [Figure 15]b isolated LPA stenosis may be caused by constriction of ductal tissue extended into LPA (post duct closure) or post -device or coil closure of duct (over-sized device or device/coil migration). Measurement of RV systolic pressure (TR jet velocity) is the best echo method to assess the severity of PPS when diffuse and distal narrowing is present. | Figure 15: Peripheral pulmonary stenosis. (a) Second review of suspected case of Alagille syndrome small branch pulmonary arteries bilaterally. And persistent turbulent flow. (b) Physiological peripheral PS in newborn (murmur disappeared at 6 months of age). PS: Pulmonary stenosis
Click here to view |
| Physiological Peripheral Pulmonary Stenosis of Neonate - Innocent Murmurs of Neonates | | |
Chatelain et al. did echo-study in 31 neonates (21 with the murmur-case group and 10 without murmur-control group) to find out the determinants of transient murmur.[45] They found 90% of these babies have relatively smaller diameter of branch PA's and had relatively higher Doppler velocities.[45] In 3 months, the murmur either disappeared (14/21) or diminished (9/21). None of them advanced to PPS. In our own experience, the majority of neonates presenting with a systolic murmur and increased Doppler velocity in one or both branch PA, get rid of murmur in 3–6 months of period and show normal velocity on echo-evaluation.
| Acquired Pulmonary Stenosis | | |
Acquired PS is a name given to conditions which lead to flow acceleration or definable gradient across the RVOT mostly caused by any type of external compression by pericardial bands, tumors, aneurysmal dilatation of adjacent structure etc.[36],[46] Similar kinds of flow acceleration can be seen with intracardiac tumors (rhabdomyoma or metastasis), intracardiac goiter, thrombus or big vegetations. The identification of etiology of RVOT gradient must be identified correctly and managed accordingly.
| Conclusion | | |
Isolated PS is a CHD that can be managed with timely interventions with minimum mortality and morbidity. Echo-evaluation includes morphological echo-based detailing of lesion or lesions, evaluation of volume and function of the right ventricle, quantitative assessment of pulmonary and TR, functional assessment of left ventricle and discovering other associated cardiac abnormalities. However, a subset of PS may have associated RV hypoplasia and need to be identified as early as possible. Early recognition and timely fetal or postnatal intervention remain the key to the optimum result. Long-term monitoring is required to monitor function, PR, TR, recurrence of PS or development of other complications like subaortic stenosis. An adequate echo-evaluation is pivotal to the best outcome.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
| References | | |
| 1. | Snider AR, Serwer GA, Ritter AB. Abnormalities of ventricular outflow. In: Echocardiography in Pediatric Heart Disease. Ch. 10., 2 nd ed. St Louis: Mosby-Year Book Inc.; 1997. p. 408-51.  |
| 2. | Tinker J, Howitt G, Markman P, Wade EG. The natural history of isolated pulmonary stenosis. Br Heart J 1965;27:151-60. |
| 3. | Baumgartner H, Hung J, Bermejo J, Chambers JB, Evangelista A, Griffin BP, et al. Echocardiographic assessment of valve stenosis: EAE/ASE recommendations for clinical practice. J Am Soc Echocardiogr 2009;22:1-23. |
| 4. | Saxena A, Relan J, Agarwal R, Awasthy N, Azad S, Chakrabarty M, et al. Indian guidelines for indications and timing of intervention for common congenital heart diseases: Revised and updated consensus statement of the Working group on management of congenital heart diseases. Ann Pediatr Cardiol 2019;12:254-86. |
| 5. | Gómez-Montes E, Herraiz I, Mendoza A, Albert L, Hernández-García JM, Galindo A. Pulmonary atresia/critical stenosis with intact ventricular septum: Prediction of outcome in the second trimester of pregnancy. Prenat Diagn 2011;31:372-9. |
| 6. | Hoffman JI, Kaplan S. The incidence of congenital heart disease. J Am Coll Cardiol 2002;39:1890-900. |
| 7. | Perloff J, Marelli A. Pulmonary stenosis with interatrial communication. In: Perloff's Clinical Recognition of Congenital Heart Disease. 6 th ed.., Ch. 11. Philadelphia, PA: W.B. Saunders Ltd.; 2012. |
| 8. | Ko JM. Genetic syndromes associated with congenital heart disease. Korean Circ J 2015;45:357-61. |
| 9. | Poaty H, Mbika-Cardorelle A, Moukouma C, Mouko AA. Clinical diagnosis of noonan syndrome and brief review of literature. Ann Med Health Sci Res 2017;7:76-795. |
| 10. | Walker LA, Buttrick PM. The right ventricle: Biologic insights and response to disease: Updated. Curr Cardiol Rev 2013;9:73-81. |
| 11. | Ho SY, Nihoyannopoulos P. Anatomy, echocardiography, and normal right ventricular dimensions. Heart 2006;92 Suppl 1:i2-13. |
| 12. | Sholler GF, Colan SD, Sanders SP. Lower values for cardiac index, indexed LV end-diastolic volume, ejection fraction, ratio of early to atrial LV diastolic inflow. Am J Cardiol 1988;62:778-84. |
| 13. | Beçu L, Somerville J, Gallo A. “Isolated” pulmonary valve stenosis as part of more widespread cardiovascular disease. Br Heart J 1976;38:472-82. |
| 14. | Gikonyo BM, Lucas RV, Edwards JE. Anatomic features of congenital pulmonary valvar stenosis. Pediatr Cardiol 1987;8:109-16. |
| 15. | Leblanc MH, Paquet M. Echocardiographic assessment of valvular pulmonary stenosis in children. Br Heart J 1981;46:363-8. |
| 16. | Rodbard S, Ikeda K, Montes M. An analysis of mechanisms of post stenotic dilatation. Angiology 1967;18:349-67. |
| 17. | Geggel RL. Ebstein's anomaly associated with severe valvar pulmonary stenosis: Successful palliation with balloon pulmonary valvuloplasty in an adult. Catheter Cardiovasc Interv 1999;46:441-4. |
| 18. | Galindo A, Gutiérrez-Larraya F, Velasco JM, de la Fuente P. Pulmonary balloon valvuloplasty in a fetus with critical pulmonary stenosis/atresia with intact ventricular septum and heart failure. Fetal Diagn Ther 2006;21:100-4. |
| 19. | Rao PS. Doppler ultrasound in the prediction of transvalvar pressure gradients in patients with valvar pulmonary stenosis. Int J Cardiol 1987;15:195-203. |
| 20. | Williams JC, Barratt-Boyes BG, Lowe JB. Underdeveloped right ventricle and pulmonary stenosis. Am J Cardiol 1963;11:458-68. |
| 21. | Singhal A, Kumar S, Kapoor A. Sudden iatrogenic suicidal right ventricle. Indian Heart J 2015;67:406-8. |
| 22. | Hanley FL, Sade RM, Blackstone EH, Kirklin JW, Freedom RM, Nanda NC. Outcomes in neonatal pulmonary atresia with intact ventricular septum. A multiinstitutional study. J Thorac Cardiovasc Surg 1993;105:406-23, 424-7. |
| 23. | Schneider M, Aschauer S, Mascherbauer J, Ran H, Binder C, Lang I, et al. Echocardiographic assessment of right ventricular function: Current clinical practice. Int J Cardiovasc Imaging 2019;35:49-56. |
| 24. | Snider AR, Serwer GA, Ritter AB. Methods for obtaining quantitative information from echocardiographic examination. In: Echocardiography in Pediatric Heart Disease. 2 nd ed.., Ch. 5. Mosby, St Louis: Mosby-Year Book Inc.; 1997. p. 408-51. |
| 25. | Dwivedi A, Axel L. Abnormal motion patterns of the interventricular septum. JACC Cardiovasc Imaging 2017;10:1281-4. |
| 26. | Anavekar NS, Gerson D, Skali H, Kwong RY, Yucel EK, Solomon SD. Two-dimensional assessment of right ventricular function: An echocardiographic-MRI correlative study. Echocardiography 2007;24:452-6. |
| 27. | Hoette S, Creuzé N, Günther S, Montani D, Savale L, Jaïs X, et al. RV fractional area change and TAPSE as predictors of severe right ventricular dysfunction in pulmonary hypertension: A CMR study. Lung 2018;196:157-64. |
| 28. | Márquez-González H, Vargas MH, Yáñez-Gutiérrez L, Almeida-Gutiérrez E, Garduño-Espinosa J. Tei index is the best echocardiographic parameter for assessing right ventricle function in patients with unrepaired congenital heart diseases with outflow tract obstruction. Front Pediatr 2018;6:181. |
| 29. | Lange PE, Onnasch DG, Heintzen PH. Valvular pulmonary stenosis: Natural history and right ventricular function. Eur Heart J 1985;6:706-9. |
| 30. | Gielen H, Daniëls O, Van Lier H. Natural history of congenital pulmonary valvar stenosis: An echo and Doppler cardiographic study. Cardiol Young 1999;9:129-35. |
| 31. | Nagueh SF, Kopelen HA, Zoghbi WA. Relation of mean right atrial pressure to echocardiographic and Doppler parameters of right atrial and right ventricular function. Circulation 1996;93:1160-9. |
| 32. | Minich LL, Tani LY, Shaddy RE, Snider AR. Doppler systemic venous flow patterns: Changes in children with mild/moderate pulmonic stenosis. J Am Soc Echocardiogr 1996;9:814-8. |
| 33. | Lueker RD, Vogel JH, Blount SG Jr. Regression of valvular pulmonary stenosis. Br Heart J 1970;32:779-82. |
| 34. | Agha H, El-Saeidi S, Abou Seif H, Abd El-Salam M, El Amrousy D. Right ventricular growth and function after balloon valvuloplasty for critical pulmonary valve stenosis in infants and neonates. Pediatr Cardiol 2020;41:795-800. |
| 35. | Mohanty S, Pandit BN, Tyagi S. Balloon pulmonary valvotomy not just a simple balloon dilatation. Indian Heart J 2014;66:462-5. |
| 36. | Harrild DM, Powell AJ, Tran YX, Geva T, Lock JE, Rhodes J, et al. McElhinney: Long-term pulmonary regurgitation following balloon valvuloplasty for pulmonary stenosis: Risk factors and relationship to exercise capacity and ventricular volume and function. J Am Coll Cardiol 2010;55:1041-7. |
| 37. | Steinberger J, Moller JH. Exercise testing in children with pulmonary valvar stenosis. Pediatr Cardiol 1999;20:27-32. |
| 38. | Krabill KA, Wang Y, Einzig S, Moller JH. Rest and exercise hemodynamics in pulmonary stenosis; Comparision of children and adults. Am J Cardiol 1985;56:360-5. |
| 39. | Gasul BM, Dillon RF, Vrla V, Hait G. Ventricular septal defects; their natural transformation into the cyanotic or noncyanotic type of tetralogy of Fallot. J Am Med Assoc 1957;164:847-53. |
| 40. | Dryerre HW, Walmsley R. Stenosis at the lower bulbar orifice of infundibulum. Br Heart J 1939;1:325. |
| 41. | Cabrera A, Martinez P, Rumoroso JR, Alcibar J, Arriola J, Pastor E, et al. Double-chambered right ventricle. Eur Heart J 1995;16:682. |
| 42. | Lucas RV Jr., Marshall RJ, Morgan DZ, Warden HE. Anomalous muscle bundle of the right ventricle with intact ventricular septum: A newly recognized cause of right ventricular obstruction. Circulation 1963;28:759. |
| 43. | Yuan SM. Supravalvular pulmonary stenosis: Congenital versus acquired. Acta Med Mediterr 2017;33:849-56. |
| 44. | Gay B Jr., Shunford WH, Rogers JV Jr. The roentgenologic features of single and multiple coarctations of the pulmonary artery and branches. Am J Roentgenol Radium Ther Nucl Med 1963;90:99-613. |
| 45. | Chatelain P, Oberhänsli I, Friedli B. Physiological pulmonary branch stenosis in newborns: 2D-echocardiographic and Doppler characteristics and follow up. Eur J Pediatr 1993;152:559-63. |
| 46. | Littler WA, Meade JB, Hamilton DI. Acquired pulmonary stenosis. Thorax 1970;25:465-71. |
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8], [Figure 9], [Figure 10], [Figure 11], [Figure 12], [Figure 13], [Figure 14], [Figure 15]
[Table 1], [Table 2], [Table 3], [Table 4], [Table 5], [Table 6], [Table 7]
|
Search Pubmed for